Abrikossof's tumor Abscess Academy rash Acanthamebiasis

A

Abrikossof’s tumor

�

Granular cell tumor

Abscess

Definition

Accumulation of pus in tissue, usuallycaused by a bacterial infection

�

Furuncle

References

Lowy, FD (1998) Staphylococcus aureus infec-tions. New England Journal of Medicine 339:520–532

Academy rash

�

Erythema infectiosum

Acanthamebiasis

Synonym(s)

None

Definition

Cutaneous and/or systemic infection causedby one of several species of acanthamoeba

Pathogenesis

Opportunistic infection, most often in animmunocompromised host, particularlywith HIV disease

Clinical manifestation

Multiple pustules; infiltrated papules andplaques; subcutaneous nodules; non-heal-ing cutaneous ulcers; distribution mainlyon the extremities

Differential diagnosis

Furunculosis; disseminated varicella/zosterinfection; deep fungal infection; bacillaryangiomatosis; myctobacterial infection;pyoderma gangrenosum

Therapy

Multidrug regimen for systemic disease:pentamidine; flucytosine; fluconazole; sul-fadiazine

References

Murakawa GJ, McCalmont T, Altman J, Telang GH, Hoffman MD, Kantor GR, Berger TG (1995) Disseminated acanthamebiasis in patients with AIDS. A report of five cases and a review of the literature. Archives of Dermatology 131(11):1291–1296

PART1.MIF Wednesday, October 29, 2003 4:13 PM

2 Acanthoma fissuratum

Acanthoma fissuratum

Synonym(s)

Granuloma fissuratum

;

spectacle framegranuloma

;

acanthoma fissuratum cutis

Definition

Keratotic papule or nodule which developsat the site of chronic irritation, such asunder eye glasses or in the oral cavity

Pathogenesis

Chronic contact irritation; includes otherfactors such as local anatomic changes, seb-orrheic dermatitis, and hyperhidrosis


Oral cavity: solitary smooth-surfacedpapule at the juncture of the lip and gumFace or post-auricular fold: pink papulewith a longitudinal central fissure


Oral cavity: squamous cell carcinoma.Skin: basal cell carcinoma; foreign bodygranuloma; chondrodermatitis nodularishelicis

Therapy

Removal of stimulus by changing eyeglasses, dentures, etc.; surgical excision inrecalcitrant cases

References

Frey T, Bartak P (1992) Acanthoma supratro-chantericum. Cutis 49(6):412–416

Acanthoma fissuratum cutis

�

Acanthoma fissuratum

Acanthome à cellules claires

�

Clear cell acanthoma

Acanthosis nigricans

Synonym(s)

None

Definition

Hyperpigmented, velvety thickening of theskin; most commonly on the neck, in theaxillae, and in the groin

Pathogenesis

Caused by factors that stimulate epidermalkeratinocyte and dermal fibroblast prolifer-ation, such as insulin or an insulin-likegrowth factor


Symmetrical, hyperpigmented, velvetyplaques, which most commonly appear inthe intertriginous areas; skin tags in thevicinity of the plaques


Becker nevus; confluent and reticulatedpapillomatosis of Gougerot and Carteaud;Dowling-Degos disease; seborrheic kerato-sis; ichthyosis hystrix; linear epidermalnevus; parapsoriasis en plaque; pemphigusvegetans; hemochromatosis; Addison’s dis-ease; pellagra

Therapy

Correction of underlying disease process;weight reduction in obese patients; tretin-oin 0.025% cream; adapalene 0.1% gel; cal-cipotriene; dietary fish oils; dermabrasion

References

Hud JA Jr, Cohen JB, Wagner JM, Cruz PD Jr (1992) Prevalence and significance of acantho-sis nigricans in an adult obese population. Arch Dermatol 128: 941–944

Accessory nipples

�

Supernumerary nipple


Acinetobacter infection 3

A

Accessory tragus

Synonym(s)

Supernumerary ear

;

supernumerary auri-cle

;

accessory external ear

;

rudimentaryauricle

;

accessory auricle

;

auricularappendage

;

cervical auricle

;

preauricularappendage

;

cutaneous cervical tag

;

preauricular appendage

;

wattle

Definition

Congenital anomaly of branchial archdevelopment, producing a preauricularpapule

Pathogenesis

Abnormal development of portions of oneof the branchial arches


Asymptomatic, solitary, flesh-coloredpapule, usually in the preauricular area; vel-lus hairs arise from the papule


Preauricular cyst or sinus; thyroglossal ductcyst; branchial cyst or sinus; bronchogeniccyst; acrochordon; melanocytic nevus; epi-dermoid cyst; neurofibroma

Therapy

Surgical excision

References

Jansen T; Romiti R; Altmeyer P (2000) Accessory tragus: report of two cases and review of the lit-erature. Pediatric Dermatology 17:391–394

Accutane

�

Isotretinoin

Acetowhite test

Synonym(s)

None

Definition

Application of 3% acetic acid to lesions sus-picious for human papillomavirus infec-tion; positive test indicated by lesion turn-ing white

References

Kitchener HC, Symonds P (1999) Detection of cervical intraepithelial neoplasia in developing countries. Lancet 353:869–873

Achromic nevus

�

Nevus depigmentosus

Acinetobacter infection

Synonym(s)

None

Definition

Infection caused by Acinetobacter, a gramnegative organism

Accessory tragus.

Solitary preauricular flesh-colored papule


4 Acitretin

Pathogenesis

Opportunistic infection from an organismwhich is often a part of the normal flora inthe axilla and groin; increased sweatingresulting in higher carriage levels; skininvolvement usually colonization ratherthan infection


No physical findings in colonized patients;skin pustules, cellulitis with clinical infec-tion


Other gram negative infections; ecthyma;staphyloccal cellulitis

Therapy

No therapy for colonization; treatment ofactive infection dependent on sensitivitiesof the organism in the individual patient

References

Cunha BA, Klein NC (1995) Pseudoinfections: a review. Infectious Disease Clinical Practice 4:95–103

Acitretin

Trade name(s)

Soriatane

Generic available

No

Drug class

Retinoid

Mechanism of action

Induction of cellular differentiation; anti-inflammatory; anti-proliferative

Dosage form

10 mg, 25 mg capsule

Dermatologic indications and dosage

See table

Common side effects

Cutaneous:

cheilitis, sticky skin, alopecia,dry skin, pruritus, paronychia, desquama-tion of hands and feet

Laboratory:

hyperlipidemia

Musculoskeletal:

myalgias; arthralgias

Ocular:

dry eyes

Serious side effects

Gastrointestinal:

pancreatitis, hepatotoxic-ity

Miscellaneous:

major birth defects

Musculoskeletal:

spinal hyperostosis

Neurologic:

pseudotumor cerebri

Drug interactions

Norethindrone; methotrexate

Other interactions

Alcohol

Contraindications/precautions

Hypersensitivity to drug class or compo-nent; pregnancy; renal or hepatic dysfunc-tion; children may be more sensitive to thedrug’s effect on bones, which may preventnormal bone growth during puberty

References

Katz HI, Waalen J, Leach EE (1999) Acitretin in psoriasis: an overview of adverse effects. Jour-nal of the American Academy of Dermatology 41(3 Pt 2):S7–S12

Ackerman tumor

�

Verrucous carcinoma

Ackerman’s tumor

�

Verrucous carcinoma


Ackerman’s tumor 5

A

Acitretin. Dermatologic indications and dosage

Disease Adult dosage Child dosage

Balanitis xerotica obliterans

25–50 mg PO daily as a single dose; after four weeks, 25–75 mg PO daily

10–25 mg PO daily

Berardinelli-Seip syndrome

75 mg PO daily 10–25 mg PO daily

Darier disease 25–50 mg PO daily as a single dose; after four weeks, 25–75 mg PO daily

10–25 mg PO daily

Epidermolytic hyperkeratosis

0.5–1 mg per kg PO daily indefinitely

0.5 mg per kg PO daily indefinitely

Erythrokeratodermia variabilis

25–50 mg PO daily indefinitely 10–25 mg PO daily

Graft-versus-host disease

1 mg per kg PO daily 10–25 mg PO daily

Hairy tongue 25–50 mg daily for up to 5 months 10–25 mg PO daily

Harlequin ichthyosis 1 mg per kg PO daily 1 mg per kg PO daily indefinitely

Hidradenitis suppurativa

1 mg per kg PO daily for 4–8 months 10–25 mg PO daily

Hyperkeratosis lenticularis perstans


Kyrle’s disease 1 mg per kg PO daily for 4–8 months 10–25 mg PO daily

Lamellar ichthyosis 1 mg per kg PO daily 10–25 mg PO daily

Lichen planus 25–50 mg PO daily as a single dose; after four weeks, 25–75 mg PO daily

10–25 mg PO daily

Lichen sclerosus 25–50 mg PO daily as a single dose; after four weeks, 25–75 mg PO daily

10–25 mg PO daily

Lipoid proteinosis 25–50 mg daily for up to 5 months 10–25 mg PO daily

Lupus erythematosus 25–50 mg PO daily as a single dose; after four weeks, 25–75 mg PO daily

10–25 mg PO daily

Mal de Meleda 25–50 mg PO daily as a single dose; after four weeks, 25–75 mg PO daily

10–25 mg PO daily

Nevus verrucosus 25–50 mg daily for up to 5 months 10–25 mg PO daily

Olmsted syndrome 1 mg per kg PO daily 10–25 mg PO daily

Pachonychia congenita


10–25 mg PO daily

Palmoplantar keratoderma


10–25 mg PO daily

Papillon-Lefévre syndrome


10–25 mg PO daily

Papular mucinosis 1 mg per kg PO daily 10–25 mg PO daily

Pityriasis rubra pilaris 25–50 mg PO daily as a single dose; after four weeks, 25–75 mg PO daily

10–25 mg PO daily

Progressive symmetric erythrokeratodermia



6 Acne aestivalis

Acne aestivalis

Synonym(s)

Mallorca acne

Definition

Monomorphous follicular papular erup-tion which occurs after sun exposure

Pathogenesis

Sun exposure appears to produce thelesions; may be a variant of polymorphouslight eruption; hypersensitivity reaction tosunscreens or cosmetics possible contribut-ing factor


Monomorphous follicular papules over theshoulders, arms, chest, and neck; no come-dones present


Folliculitis; acne vulgaris; steroid acne;insect bite reaction; polymorphous lighteruption

Therapy

Tretinoin 0.025% cream; benzoyl peroxide5% gel; prophylaxis by increasing expo-sures to artificial ultraviolet radiation to“harden” the skin to the effects of sunlight

References

Plewig G, Jansen T (1998) Acneiform dermatoses. Dermatology 196:102–107

Acne atrophica

�

Acne necrotica

Acne comedonica

Synonym(s)

Comedonal acne; blackheads; whiteheads

Definition

Open and closed comedones on the face,chest, and back

Pathogenesis

Accumulation of corneocytes in the follicu-lar infundibulum, producing a sphericaldermal papule (see acne vulgaris); causeunknown but may involve stimulation ofthe follicular lining and sebaceous duct byexogenous compounds, an endogenoushormonal stimulus, or a neurologic stimu-lus

Psoriasis 25–50 mg PO daily as a single dose; after four weeks, 25–75 mg PO daily

10–25 mg PO daily

Reiter syndrome 25–50 mg PO daily as a single dose; after four weeks, 25–75 mg PO daily

10–25 mg PO daily

Striate keratoderma 0.5–1 mg per kg daily indefinitely 10–25 mg PO daily

Subcorneal pustular dermatosis

1 mg per kg PO daily 10–25 mg PO daily

Tyrosinemia II 0.5–1 mg per kg daily indefinitely 10–25 mg PO daily

Vohwinkel’s syndrome 25–50 mg PO daily as a single dose; after four weeks, 25–75 mg PO daily

10–25 mg PO daily

Acitretin. Dermatologic indications and dosage (Continued)



Acne excoriée 7

A


Open comedone: skin-colored or white,slightly elevated papule with a punctatecentral openingClosed comedone: slightly raised papulewith a central black keratotic plug


Milium; epidermoid cyst; giant pore ofWiner; nevus comedonicus; Favre-Racou-chot disease; radiation acne; acne cosmet-ica; chloracne; trichostasis spinulosa; flatwarts; appendageal tumors (syringoma,etc.); sebaceous gland hyperplasia

Therapy

Tretinoin cream 0.025%

�

; tazarotene 0.1%;adapalene 0.1% gel

�

; benzoyl peroxide 5%gel; azelaic acid 20% cream; salicylic acid 1–2% cream or gel; alpha hydroxy acid prepa-ration; trichloroacetic acid 10–20% peel

References

Webster, GF (1999) Acne vulgaris. Archives of Dermatology 135:1101–1102

Acne conglobata

Synonym(s)

Conglobate acne

DefinitionInflammatory disease characterized bycysts, double-headed comedones, abscesses,sinus tracts, and severe scarring; occursalmost exclusively in adult men

PathogenesisUnknown

Clinical manifestationNumerous large comedones with multipleopenings; multiple inflammatory papules,pustules, nodules, and cysts; distribution oflesions over back, chest, buttocks, arms,abdomen, and thighs; heals with deep pit-ted scars and hypertrophic scars

Differential diagnosisAcne inversa; acne fulminans; chloracne;tropical acne

TherapyIsotretinoin�; prednisone for extreme acuteflares; dapsone; incision and drainage ofsuppurative cysts and nodules; triamci-nolone 3–5 mg per ml intralesional toinflamed cysts; liquid nitrogen cryotherapyfor hemorrhagic nodules; surgical excisionand skin grafting of chronically involvedsites

ReferencesChicarilli ZN (1987) Follicular occlusion triad:

hidradenitis suppurativa, acne conglobata, and dissecting cellulitis of the scalp. Annals of Plas-tic Surgery 18:230–237

Acne decalvans

� Folliculitis decalvans

Acne excoriée

Synonym(s)Picker’s acne; excoriated acne

DefinitionAcne lesions which are excoriated

PathogenesisSelf-induced lesions, often in patientswhose acne becomes a source of extrememental distress

Clinical manifestationIrregular crusts at sites of acne which havebeen excoriated

Differential diagnosisAtopic neurodermatitis; depression withself-mutilation; ecthyma; herpes simplexvirus infection


8 Acne frontalis

TherapyTreatment of underlying acne (see acne vul-garis); discussion of the cause of the excori-ations; psychotherapy in selected patients

ReferencesArnold LM, Auchenbach MB, McElroy SL (2001)

Psychogenic excoriation. Clinical features, pro-posed diagnostic criteria, epidemiology and approaches to treatment. CNS Drugs 15:351–359

Acne frontalis

� Acne necrotica

Acne inversa

� Hidradenitis suppurativa

Acne keloid

� Acne keloidalis

Acne keloidalis

Synonym(s)Acne keloidalis nuchae; folliculitis keloida-lis; folliculitis keloidalis nuchae; acne keloid

DefinitionChronic inflammatory process of the hairfollicles leading to keloidal papules andplaques on the occipital scalp and posteriorneck

PathogenesisTheories: injury produced by short hair-cuts; irritation from shirt collars; chroniclow-grade bacterial infections; autoim-mune process; primary scarring alopecia;weakened follicular wall with subsequentrupture and foreign body reaction

Clinical manifestationFirm, dome-shaped, follicular papules,which develop on the nape of the neck and/or on the occipital scalp; papules coalesceinto plaques; scarring alopecia and subcuta-neous abscesses with draining sinusesoccur later in the course

Differential diagnosisFolliculitis; acne vulgaris; perifolliculitiscapitis abscedens et suffodiens; nevus seba-ceous of Jadassohn; keloid; pediculosiscapitis; hidradenitis suppurativa; sebor-rheic dermatitis; squamous cell carcinoma;basal cell carcinoma

TherapyAvoidance of trauma to the neck and poste-rior scalp area; triamcinolone (5–10 mg perml) intralesional after softening the sitewith light liquid nitrogen cryotherapy; CO2laser vaporization followed by intralesionaltriamcinolone (5–10 mg per ml) or imiqui-mod 5% cream applied daily for 6–8 weeks;punch excision of individual papules; hori-zontal ellipical excision with or without pri-mary closure

ReferencesGloster HM Jr (2000). The surgical management

of extensive cases of acne keloidalis nuchae. Ar-chives of Dermatology 136:1376–1379

Acne keloidalis nuchae

� Acne keloidalis


Acne necrotica miliaris 9

AAcne medicamentosa

Synonym(s)None

DefinitionAcneiform eruption related to ingestion of amedication

PathogenesisUnknown; not an allergic reaction to themedication; not a variant of acne vulgaris

Clinical manifestationAcute onset of inflammatory papules in thethe same stage of development with few orno comedones; occurs on the chest, back,and upper extremities; causative agentsinclude systemic corticosteroids, anabolicsteroids, B vitamins, anticonvulsants, lith-ium, isoniazid, quinidine, azathioprine,cyclosporine, etretinate, and halides

Differential diagnosisAcne vulgaris; folliculitis; chloracne; tropi-cal acne; acne aestavalis

TherapyDiscontinue offending medication, if possi-ble; tetracycline; tretinoin 0.025% cream

ReferencesWebster, GF (2002) Acne. British Medical Journal

325:475–479

Acne necrotica

Synonym(s)Acne necrotica miliaris; acne variolafor-mis; acne frontalis; acne atrophica;necrotizing lymphocytic folliculitis;pustular perifolliculitis

DefinitionPapulopustular follicular eruption whichheals with depressed scars

PathogenesisGenetic factors possibly operative

Clinical manifestationRecurrent grouped perifollicular papulesand pustules which heal with variolaformscars; most often located in the temporalscalp, but also on the face, chest, and back

Differential diagnosisBacterial folliculitis; tinea capitis; vasculi-tis; papulonecrotic tuberculid; hydroa vac-ciniforme

TherapyTetracycline; isotretinoin 1 mg per kg POcombined with prednisone 1 mg per kg perday PO�; antibacterial washes with chlo-rhexadine or hexachlorophene 2–3 timesdaily; daily shampooing

ReferencesKossard S, Collins A, McCrossin I (1987) Necrotiz-

ing lymphocytic folliculitis: the early lesion of acne necrotica. Journal of the American Acade-my of Dermatology 16:1007–1014

Acne necrotica miliaris

� Acne necrotica

Acne Medicamentosa. Monomorphous red papules on the arm and lateral chest wall


10 Acne rosacea

Acne rosacea

� Rosacea

Acne variolaformis

� Acne necrotica

Acne varus

� Acne vulgaris

Acne vulgaris

Synonym(s)Acne varus

DefinitionCommon, self-limited eruption character-ized by abnormal follicular keratinization,comedones, inflammatory papules, pus-tules, and nodular abscesses

PathogenesisMultiple contributing factors includinginheritance, hormonal effects on follicles,increased sebum production, bacteria,abnormal follicular keratinization, andresponse to environmental stimuli such asoils and frictional trauma

Clinical manifestationClosed comedones (whitehead); opencomedones (blackhead); inflammatorypapules and pustules; nodules; drainingsinuses; postinflammatory scars; lesions inareas with abundant sebaceous follicles:face, back, upper chest wall

Differential diagnosisAcne aestivalis; rosacea; perioral dermati-tis; folliculitis; acne medicimentosa; occu-pational acne; tropical acne; acne cosmet-ica; syndrome of Favre-Racouchot; flatwarts; trichostasis spinulosa

TherapyComedonal acne: tretinoin 0.025% cream oradapalene 0.1% gel or tazarotene 0.1% gel;alpha hydroxy acid preparationInflammaroty acne: tetracycline or doxycy-cline or minocycline; benzoyl peroxide 5%gel; azelaic acid 20% cream; clindamycin 1%lotion or cream; erythromycin 2% gel orcreamRecalcitrant acne in women: oral contracep-tive containing norgestimate 0.25 mg andethinyl estradiol 0.035 mg; spironolactone;prednisoneAcne where sweating is an aggravating fac-tor: aluminium chloride solutionSevere nodulocystic acne unresponsive toother therapies: isotretinoin�

Acne surgery: comedone expression; inci-sion and drainage of fluctuant cysts andabscesses; chemical peel; microdermabra-sion; intralesional triamcinolone 2–4 mg/ml

ReferencesWebster GF (2002) Acne vulgaris. British Medical

Journal 325:475–479

Acoustic neuroma

� Granular cell tumor

Acquired digital fibrokeratoma

Synonym(s)Garlic glove fibroma


Acquired perforating dermatitis 11

ADefinitionBenign, acquired, hyperkeratotic projec-tion, usually on one of the digits

PathogenesisTrauma possibly a contributing factor

Clinical manifestationSolitary, smooth, asymptomatic, dome-shaped, skin-colored papule with a collar-ette of skin encircling the base of thegrowth, creating a moat-like effect; lesionusually arising on one of the digits of thehand, but also occurring on the palms andsoles, dorsum of the hand, wrist, calf, toe,or pre-patellar area

Differential diagnosisWart; periungual fibroma (Koenen tumor);pyogenic granuloma; fibroma; supernumer-ary digit

TherapySimple excision�

ReferencesVinson RP, Angeloni VL (1995): acquired digital

fibrokeratoma. American Family Physician 52:1365–1367

Acquired epidermolysis bullosa

� Epidermolysis bullosa acquisita

Acquired generalized anhidrosis

Synonym(s)Tropical anhidrotic asthenia

DefinitionGeneralized loss of sweat function follow-ing prolonged sun exposure

PathogenesisUnknown

Clinical manifestationLoss of sweat function after prolongedexposure to the sun

Differential diagnosisNone

TherapyAvoidance of situations where core bodytemperature may rise (exercise, sun expo-sure, etc.)

ReferencesTsuji T, Yamamoto T (1976) Acquired generalized

anhidrosis. Archives of Dermatology 112:1310–1314

Acquired hypertrichosis

DefinitionExcess hair growth in androgen-independ-ent sites; occurs in men and women

ReferencesManders SM (1995) Acquired hypertrichosis. In:

demis DJ (ed) Clinical Dermatology. Lippincott Williams and Wilkins, Philadelphia, Section 2–27, pp 1–4

Acquired partial lipodystrophy

� Progressive lipodystrophy

Acquired perforating dermatitis

� Perforating folliculitis


12 Acquired perforating dermatosis

Acquired perforating dermatosis


Acquired perforating disease

� Reactive perforating collagenosis

Acquired progressive lipodystrophy


Acquired reactive perforating dermatosis


Acquired tufted angioma

� Tufted angioma

Acral lentiginous melanoma

Synonym(s)Acral melanoma

DefinitionMelanoma affecting the palms, soles, sub-ungual, and periungual skin or the mucousmembranes

PathogenesisUnknown

Clinical manifestationSubungual melanoma: diffuse nail discolor-ation or a longitudinal pigmented bandwithin the nail plate, with bleeding of pig-ment onto the nail fold (Hutchinson’s sign)Palmer or plantar melanoma: irregularlypigmented plaque with variable nodularityand late erosion or ulcerationMucosal melanoma: unevenly pigmentedmacule, patch, or plaque, with an asymmet-ric shape and irregular borders and surface

Differential diagnosisLentigo; subungual hematoma; chronic par-onychia; nevus; melanonychia striata;benign mucosal melanosis; traumatic tat-too; Kaposi’s sarcoma; pyogenic granuloma

TherapySee melanoma

ReferencesRogers RS 3rd, Gibson LE (1997) Mucosal, genital,

and unusual clinical variants of melanoma. Mayo Clinic Proceedings 72:362–366

Acral melanoma

� Acral lentiginous melanoma

Acral persistent papular mucinosis

Synonym(s)None

DefinitionChronic localized papular mucinous erup-tion of the upper extremities

PathogenesisUnknown


Acrocephalosyndactyly 13

AClinical manifestationMultiple, discrete, flesh-colored or ivory-colored papules of the hands, wrists, andforearms; occurs in middle-aged women;not associated with systemic findings

Differential diagnosisCutaneous focal mucinosis; lupus ery-thematosus; mucocoele; digital mucouscyst; reticular erythematous mucinosis;cutaneous myxoma; urticarial follicularmucinosis

TherapyNone

ReferencesFlowers SL, Cooper PH, Landes HB (1989) Acral

persistent papular mucinosis. Journal of the American Academy of Dermatology 21:293–297

Acroangiodermatitis

Synonym(s)Pseudo Kaposi’s sarcoma; Mali’s disease;acroangiodermatitis of Mali; angiodermitéde Favre et Chaix; Favre-Chaix disease;Stewart-Bluefarb syndrome

DefinitionHyperplasia of preexisting vasculature inpatients with chronic venous insufficiency

PathogenesisSevere chronic venous stasis and insuffi-ciency of the calf muscle pump resulting inan elevated capillary pressure; insufficiencyof both the muscular pump of the calf andthe venous pump of the foot, producing rel-ative tissue anoxia which may cause sec-ondary vascular proliferation

Clinical manifestationBlue or purple papules and nodules occur-ring in chronically edematous skin; may beassociated with other signs of venous insuf-ficiency, such as varicose veins, elephantia-sis nostra, and leg ulcers

Differential diagnosisKaposi’s sarcoma; pigmented purpuric der-matosis; lichen planus; hemangioma; vas-culitis

TherapyTreatment of underlying vascular insuffi-ciency: support hose; sequential compres-sion device; Unna boots; leg elevation;weight loss; exercise programSurgical therapy: excision of individuallesions

ReferencesPires A, Depairon M, Ricci C (1999) Effect of com-

pression therapy on a pseudo-Kaposi sarcoma. Dermatology 198:439–441

Acroangiodermatitis of Mali

� Acroangiodermatitis

Acrocephalosyndactyly

Synonym(s)Apert’s syndrome; Pfeiffer’s syndrome;Saethre-Chotzen syndrome

DefinitionTower skull deformity; facial peculiarities;syndactyly of the hands and feet; increasedincidence of mental retardation

PathogenesisGenetic defect (autosomal dominant);localized mutations of FGFR2 gene

Clinical manifestationApert’s syndrome: high peaked or conicalskull; flattened face; hypertelorism; poorvision; low set ears with poor hearing acu-ity; severe syndactyly; mitten hand deform-ity; severe acne vulgarisPfeiffer’s syndrome: similar to Apert’s syn-drome, but less severe


14 Acrochordon

Saethre-Chotzen syndrome: similar toApert’s syndrome, but less severe; dentaldefects; often normal intelligence

Differential diagnosisAcrocephalopolysyndactyly syndromes;Rubinstein-Taybi syndrome; D1 trisomy;hereditary brachymegalodactyly; Léri’s ple-onostenosis

TherapyReconstructive skull surgery; isotretinoinfor severe acne vulgaris�

ReferencesPark WJ, Theda C, Maestri NE, Meyers GA, et al.

(1995) Analysis of phenotypic features and FGFR2 mutations in Apert syndrome. Ameri-can Journal of Human Genetics 57:321–328

Acrochordon

Synonym(s)Skin tag; soft wart; fibroepithelial polyp

DefinitionTumor of loose fibrous tissue, occurringmostly on the neck and in flexural areas

PathogenesisFrequent irritation; obesity; epidermalgrowth factor (EGF) and α-tissue growthfactor (TGF) possibly involved; hormoneimbalances, such as that seen in pregnancyor acromegaly possibly facilitating growth

Clinical manifestationRound, soft, pedunculated papules, whichare either flesh-colored or hyperpigmented

Differential diagnosisWart; neurofibroma; seborrheic keratosis,particularly the dermatosis papulosa nigravariety; melanocytic nevus; melanoma;fibroepithelioma of Pinkus; pseudosarco-matous polyp

TherapyScissors excision; liquid nitrogen cryother-apy; destruction by electrodesiccation

ReferencesHood AF. Lumadue J (1992) Benign vulvar tu-

mors. Dermatologic Clinics 10:371–385

Acrocyanosis

Synonym(s)None

DefinitionPersistent dusky discoloration and cool-ness of the hands and feet

PathogenesisDecreased basal flow through the acralcutaneous microcirculation; theories ofcausation: defective arteriolar physiology;blood viscosity abnormalities; elevatedendothelin-1 levels and exaggeratedresponses of this molecule to cold stimula-tion

Clinical manifestationViolaceous discoloration of the distalextremities; nose, lips, nipples, and earspossibly also involved; worsens with coldexposure; may be associated with coldagglutinin disease, cryoglobulinemia, cer-tain medications, malignancies, and infec-tions

Differential diagnosisChilblains; livedo reticularis; Raynaud phe-nomenon; erythromelalgia; lupus ery-thematosus; scleroderma

TherapyProtection of acral areas of the body fromthe cold; minoxidil 5% solution; bromocrip-tine; nicotinic acid; biofeedback training

ReferencesNousari HC, Kimyai-Asadi A, Anhalt GJ (2002)

Chronic idiopathic acrocyanosis. Journal of the


Acrodermatitis enteropathica 15

AAmerican Academy of Dermatology 45:S207–208

Acrodermatitis chronica atrophicans

Synonym(s)Chronic atrophic acrodermatitis; Lyme bor-reliosis, late phase

DefinitionFibrosing skin process due to the effect ofcontinuing active infection with Borreliaafzelii

PathogenesisSeveral nonspecific reactions with a spe-cific immune response possibly contribut-ing to its manifestations; progressive,restricted pattern of cytokine expression,including deficient interferon-γ, possiblycontributing to its chronicity

Clinical manifestationInsidious onset of reddish-brown plaquesand nodules on the distal extremities;lesions expanding outward with resultantcentral atrophy

Differential diagnosisMorphea; venous insufficiency; lichen scle-rosus et atrophicus; eosinophilic fasciitis;pernio; endemic syphilis

TherapyAbsence of signs of systemic disease: doxy-cyline�; amoxicillin.Signs and symptoms of systemic disease:ceftriaxone 2 g IV every 24 hours for 14–21days; cefotaxime 1–2 g IV every 8 hours for14–21 days; penicillin G 3–4 million units IVevery 4 hours for 21 days

ReferencesMelski JW (2000) Lyme borreliosis. Seminars in

Cutaneous Medicine & Surgery 19:10–18

Acrodermatitis enteropathica

Synonym(s)Acrodermatitis enteropathica; Danbolt-Closs syndrome; acrodermatitis entero-pathica-like syndrome; transient symptom-atic zinc deficiency; iatrogenic acrodermati-tis enteropathica; zinc deficiency syn-drome; zinc depletion syndrome; self-limiting acrodermatitis enteropathica

DefinitionAutosomal recessive disorder with skinlesions, diarrhea, alopecia, photophobia,irritability, and failure to thrive

PathogenesisDeficient intestinal absorption of zinc fromthe small intestine

Clinical manifestationSigns and symptoms appearing shortlyafter discontinuation of breast-feeding; redpatches, scaly plaques, and eczematous skinthat may evolve into crusted, vesiculobul-lous, erosive, and pustular plaques; distri-bution in a periorificial and acral pattern,on the face, scalp, hands, feet, and anogeni-tal areas; alopecia of the scalp and eye-brows; secondary staphylococcal and candi-dal skin infections

Differential diagnosisBiotin and multiple decarboxylase deficien-cies; essential fatty acid deficiencies; Lang-erhans cell histiocytosis; cystic fibrosis;mucocutaneous candidiasis; glucagonomasyndrome; seborrheic dermatitis; atopicdermatitis

TherapyZinc dietary supplementation 1 mg per kgper day for life

ReferencesRadja N, Charles-Holmes R (2002) Acrodermati-

tis enteropathica: lifelong follow-up and zinc


16 Acrodermatitis enteropathica-like syndrome

monitoring. Clinical & Experimental Derma-tology 27:62–63

Acrodermatitis enteropathica-like syndrome

� Acrodermatitis enteropathica

Acrodermatitis of Dore

� Psoriasis

Acrodermatitis papulosa

� Gianotti-Crosti syndrome

Acrodermatitis papulosa eruptiva infantilis


Acrodermatitis papulosa infantum


Acrodynia

Synonym(s)Pink disease

DefinitionMultisystem disease related to mercuryintoxication

PathogenesisSympathovasomotor dysfunction second-ary to mercury intoxication, perhaps on anidiosyncratic basis

Clinical manifestationPain in the hands and feet; hyperhidrosis;excess salivation; gingivitis; early tooth loss;pink discoloration of the nose and distaldigits; peripheral neuronitis; hypotonia ofthe muscles; renal insufficiency

Differential diagnosisAcrocyanosis; chilblains; acrodermatitisenteropathica; glucagonoma syndromeKawasaki disease; polio; intoxication withthallium, copper, arsenic, or gold

TherapyRemoval of source of mercury from theenvironment; DMSA (meso 2,3-dimercapto-succinic acid) used as a chelating agent;hemodialysis or peritoneal dialysis for renalinsufficiency

ReferencesGraeme KA, Pollack CV Jr (1998) Heavy metal

toxicity, Part I: arsenic and mercury. Journal of Emergency Medicine 16(1):45–56

Acroerythrokeratoderma

� Mal de Meleda

Acrogeria

Synonym(s)Gottron’s syndrome


Acrokeratoelastoidosis marginalis 17

ADefinitionPremature aging of the skin, predominatelyaffecting the distal extremities, withoutother features of premature aging

PathogenesisAutosomal recessive inheritiance; may berelated to type IV Ehlers-Danlos syndrome

Clinical manifestationDry, thin, wrinkled skin; most prominentover the distal extremities; dystrophic nails;short stature; normal life expenctancy

Differential diagnosisWerner’s syndrome (pangeria); progeria

TherapyNone

ReferencesGreally JM, Boone LY, Lenkey SG, Wenger SL,

Steele MW (1992) Acrometageria: a spectrum of “premature aging” syndromes. American Journal of Medical Genetics 44(3):334–339

Acrokeratoderma hereditarium punctatum

� Acrokeratoelastoidosis

Acrokeratoelastoidosis

Synonym(s)Acrokeratoelastoidosis marginalis; acro-keratoelastoidosis of Costa; acrokerato-derma hereditarium punctatum; hereditarypapulotranslucent acrokeratoderma

DefinitionPapular eruption which occurs on the mar-gins of the hands and feet

PathogenesisAutosomal dominant transmission in somecases

Clinical manifestationKeratotic translucent papules which ariseon the margins of the hands and feet;lesions often occur in a linear distribution

Differential diagnosisKeratoelastoidosis marginalis; focal acralhyperkeratosis; flat warts; acrodynia;acrokeratosis verruciformis of Hopf

TherapyTretinoin 0.025% cream

ReferencesRongioletti F, Betti R, Crosti C, Rebora A (1994)

Marginal papular acrokeratodermas: a unified nosography for focal acral hyperkeratosis, ac-rokeratoelastoidosis and related disorders. Dermatology 188(1):28–31

Acrokeratoelastoidosis marginalis


Acrokeratoelastoidosis. Confluent scaly plaques on the sides of the digits


18 Acrokeratoelastoidosis of Costa

Acrokeratoelastoidosis of Costa


Acrokeratosis paraneoplastica

� Paraneoplastic acrokeratosis

Acrokeratosis paraneoplastica of Bazex

� Paraneoplastic acrokeratosis

Acrokeratosis verruciformis

Synonym(s)Acrokeratosis verruciformis of Hopf

DefinitionAutosomal dominant disease consisting offlat wart-like papules over the dorsalaspects of the hands and feet

PathogenesisAppears to be a variant of an epithelialnevus

Clinical manifestationMultiple, asymptomatic, flesh-colored toreddish-brown, flat-topped polygonalpapules over the dorsal aspects of the handsand feet; occasional whitish discolorationand thickening of the nail plates

Differential diagnosisFlat warts; epidermodysplasia verruci-formis; stucco keratosis; lichen planus;keratosis follicularis (Darier disease);arsenical keratosis; granuloma annulare;colloid milia

TherapyDestruction with liquid nitrogen cryother-apy; CO2 laser or Nd:YAG laser; tretinoin0.025% cream; adapalene 0.1% gel

ReferencesChapman-Rolle L, DePadova-Elder SM, Ryan E,

Kantor GR (1994) Persistent flat-topped pa-pules on the extremities. Acrokeratosis verruci-formis (AKV) of Hopf. Archives of Dermatology 130(4):508–509, 511–512

Acrokeratosis verruciformis of Hopf

� Acrokeratosis verruciformis

Acromegalic gigantism (prepubertal children)

� Acromegaly

Acromegaly

Synonym(s)Hyperpituitarism; acromegalic gigantism(prepubertal children)

DefinitionA metabolic disorder caused by excessgrowth hormone that results in gradualenlargement of body tissues, including thebones of the face, jaw, hands, feet, and skull


Acropustulosis of infancy 19

APathogenesisGrowth-hormone-secreting pituitarytumors; rarely caused by ectopic growthhormone overproduction by lung or pan-creas tumors

Clinical manifestationCoarsening of facial features; darkening ofthe skin; large, spade-like hands and feet;excessive sweating; hypertrichosis; oilyskin; enlargement of the nose; thickening ofheel pads; hard and thickened nails

Differential diagnosisPachydermoperiostosis; pseudoacromega-loidism; hypothyroidism

TherapyTranssphenoidal adenomectomy; super-voltage pituitary gland radiation; octre-otide 50–500 mcg SC three time daily; bro-mocriptine 1.25 mg PO daily initially,increased gradually to 20–30 mg PO daily

ReferencesBen-Shlomo A, Melmed S (2001) Acromegaly. En-

docrinology & Metabolism Clinics of North America 30(3):565–583

Acropachy

� Clubbing of the nails

Acropapulo-vesicular syndrome


Acropigmentatio

� Reticulate Acropigmentation of Kitamura

Acropigmentation of Dohi

Synonym(s)Symmetrical dyschromatosis of the extrem-ities; acropigmentation symmetrica of Dohi

DefinitionSymmetrical, freckle-like pigmentation ofthe hands and feet, arising in early child-hood

PathogenesisAutosomal dominant inheritance

Clinical manifestationFreckle-like hyperpigmented macules onthe hands and feet; associated with hypop-igmented macules without atrophy

Differential diagnosisAcromelanosis progressiva; reticulate acro-pigmentation of Kitamura; universalacquired melanosis

TherapyNone

ReferencesDanese P, Zanca A, Bertazzoni MG (1997) Familial

reticulate acropigmentation of Dohi. Journal of the American Academy of Dermatology 37:884–886

Acropigmentation symmetrica of Dohi

� Acropigmentation of Dohi

Acropustulosis of infancy

Synonym(s)Infantile acropustulosis


20 Acrosclerosis

DefinitionPruritic vesiculopustular eruption of thepalms and soles, which occurs mostly inblack newborns and infants

PathogenesisUnknown

Clinical manifestationRecurrent crops of small vesicles whichevolve into pustules; lesions on the palms,soles, and the dorsal aspects of the distalextremities; onset between birth and 2years; spontaneous permanent remission by2–3 years of age

Differential diagnosisErythema toxicum neonatorum; dyshidro-sis; scabies; pyoderma; transient neonatalpustular melanosis; subcorneal pustulardermatosis; pustular psoriasis; cutaneouscandidiasis; fire ant bites; hand-foot-and-mouth disease; eosinophilic pustulosis

TherapyFluocinonide 0.05% cream applied twicedaily; dapsone

ReferencesWagner A (1997) Distinguishing vesicular and

pustular disorders in the neonate. Current Opinion in Pediatrics 9(4):396–405

Acrosclerosis

DefinitionThickening of the skin and subcutaneoustissue of the hands and feet due to swellingand thickening of fibrous connective tissue

ReferencesHawk A, English JC 3rd (2001) Localized and sys-

temic scleroderma. Seminars in Cutaneous Medicine & Surgery 20(1):27–37

Acrospiroma

� Eccrine acrospiroma

Acrospiroma, eccrine


Actinic cheilitis

Synonym(s)Actinic keratosis of the lip; actinic damageof the lip; solar cheilitis; actinic cheilosis

DefinitionA precancerous skin growth usually causedby chronic sun exposure to the lip

PathogenesisChronic sun exposure producing dyskera-totic cell clones which proliferate

Clinical manifestationIrregular, non-substantive scaly papule orplaque of vermillion portion of the lip

Differential diagnosisSquamous cell carcinoma; chapped lips;trauma from chronic lip licking; irritantleukoplakia secondary to cigarette smok-ing, etc.; contact dermatitis; polymorphouslight eruption; lupus erythematosus

TherapyDestruction by liquid nitrogen cryother-apy; fluorouracil cream; photodynamictherapy; laser resurfacing; dermabrasion;surgical excision with mucosal advance-ment flap


Actinic granuloma 21

AReferencesDrake LA, Ceilley RI, Cornelison RL (1995) Guide-

lines of care for actinic keratoses. Committee on Guidelines of Care. Journal of the American Academy of Dermatology 32(1):95–98

Actinic cheilosis

� Actinic cheilitis

Actinic damage of the lip


Actinic dermatitis

� Chronic actinic dermatitis

Actinic elastosis

Synonym(s)Solar elastosis; senile elastosis; dermatohe-liosis; sun damage; farmer’s neck; sailor’sneck

DefinitionHistologic degenerative changes in the skinsecondary to chronic sun exposure

PathogenesisUltraviolet-induced postinflammatory der-mal connective tissue degeneration; rela-tive contribution of UVB and UVA unclear

Clinical manifestationYellowish hue to the skin with irregular,firm papules giving the skin a chicken skin-

like appearance; dyspigmentation; redun-dant skin with deep furrows (cutis rhom-boidalis nuchae); glistening scaly plaquesalong the margins of the digits (keratoelas-toides marginalis); associated cysts andcomedones (syndrome of Favre and Racou-chot); discrete semi-translucent papules onthe antihelix or helix of the ear; annularplaques with an atrophic center (actinicgranuloma); crystalline papules filled withgelatinous material on the forearms and thetips of the ears

Differential diagnosisPapular mucinosis; pseudoxanthoma elasti-cum; polymorphous light eruption; lupuserythematosus; basal cell carcinoma; squa-mous cell carcinoma; granuloma annulare;comedonal acne; epidermoid cysts; agedskin

TherapyAvoidance of further sun damage; sun pro-tection measures such as sunscreens, pro-tective clothing; tretinoin 0.025% cream;adapalene 0.1% gel; chemical peel; laserresurfacing

ReferencesFenske NA, Hynes LR, Lober CW (1998) Actinic

elastosis (senile elastosis). In: demis DJ (ed) Clinical Dermatology. Lippincott Williams and Wilkins, Philadelphia, Section 1 4–41 pp 1–12

Actinic granuloma

Synonym(s)Miescher’s granulomatosis; annular elas-tolytic giant-cell granuloma;granulomatosis disciformis chronica etprogressiva

DefinitionChronic, plaque-like, and often annularcutaneous photoeruption, with mixedinflammatory dermal infiltrate, numerousmultinucleated giant cells, and prominentelastolysis


22 Actinic keratosis

PathogenesisUnclear whether a variant of granulomaannulare in sun-damaged skin or a sepa-rate disease entity

Clinical manifestationSlowly enlarging, asymptomatic, skin-colored or erythematous annular plaque,usually in sun-exposed skin; resolves inmonths to years without scarring

Differential diagnosisGranuloma annulare; sarcoidosis; necrobio-sis lipoidica; leprosy; syphilis; elastosis per-forans serpiginosa; lupus erythematosus;morphea

TherapyTriamcinolone 5 mg per ml intralesionally

ReferencesO'Brien JP, Regan W (1999) Actinically degenerate

elastic tissue is the likely antigenic basis of ac-tinic granuloma of the skin and of temporal ar-teritis. Journal of the American Academy of Dermatology 40(2 Pt 1):214–222

Actinic keratosis

Synonym(s)Solar keratosis; senile keratosis

DefinitionA precancerous skin neoplasm usuallycaused by chronic sun exposure

PathogenesisGenetic predisposition; occurrence morefrequent in fair, redheaded, or blondepatients that burn frequently and tanpoorly; may involve inadequate DNA repairof ultraviolet-light-induced injury

Clinical manifestationPoorly defined, red, scaly, non-substantivepapule on sun-exposed areas of the skin;occurs in the mileau of sun damage (dys-pigmentation, telangiectasia, mottling, andsolar elastosis)

Differential diagnosisSquamous cell carcinoma; seborrheic kera-tosis; wart; lichenoid keratosis; lentigomaligna; Bowen’s diseae; cutaneous lupuserythematosus

TherapyDestruction by liquid nitrogen cryotherapyor electrodesiccation and curettage; fluor-ouracil 0.5–5% cream; fluorouracil creamplus tretinoin 0.025% cream applied twicedaily for 3–6 weeks; photodynamic ther-apy; tretinoin 0.025% cream; alpha hydroxyacids; dermabrasion; chemical peel

ReferencesDrake LA, Ceilley RI, Cornelison RL (1995) Guide-

lines of care for actinic keratoses. Committee on Guidelines of Care. Journal of the American Academy of Dermatology 32(1):95–98

Actinic keratosis of the lip

� Actinic cheilitisActinic keratosis. Numerous poorly defined, red, scaly papules on the dorsal aspects of the hands


Acute febrile neutrophilic dermatosis 23

AActinic porokeratosis

� Porokeratosis

Actinic prurigo

� Polymorphous light eruption

Actinic reticuloid


Actinophytosis

� Botryomycosis

Active junctional nevus

� Atypical mole

Acute benign cutaneous leukocytoclastic vasculitis of infancy

� Acute hemorrhagic edema of infancy

Acute disseminated epidermal necrosis

� Toxic epidermal necrolysis

Acute febrile mucocutaneous lymph node syndrome

� Kawasaki disease

Acute febrile neutrophilic dermatosis

Synonym(s)Sweet syndrome; neutrophilic dermatitis

DefinitionReactive process characterized by theabrupt onset of fever and tender, red-to-purple, circinate papules, nodules, andplaques

PathogenesisHypersensitivity reaction in response tosystemic factors, which may include hema-tologic disease, infection, or drug expo-sure; neutrophil-mediated process

Clinical manifestationErythematous or violaceous papules ornodules; papules often coalescing into circi-nate or arcuate plaques; pseudovesicularappearance because of subepidermaledema; lesions occasionally studded withpustules


24 Acute generalized exanthematous pustular dermatitis

Differential diagnosisPyoderma gangrenosum; Behçet’s disease;erythema multiforme; bowel-associateddermatitis-arthritis syndrome; neutro-philic rheumatoid dermatitis; leukocyto-clastic vasculitis; leukemia cutis; cutaneousmetastasis; acute hemorrhagic edema ofchildhood

TherapyPrednisone�; steroid sparing agents: dap-sone; cyclosporine

ReferencesFett DL, Gibson LE, Su WP (1995) Sweet's Syn-

drome: systemic signs and symptoms and asso-ciated disorders. Mayo Clinic Proceedings 70:234–240

Acute generalized exanthematous pustular dermatitis

Synonym(s)Acute generalized exanthematous pustulo-sis

DefinitionGeneralized eruption of sterile pustules ondiffuse erythematous skin, shortly after theadministration of a particular drug

PathogenesisHypersensitivity reaction to drug anti-gen(s); may be a type 3 reaction

Clinical manifestationGeneralized eruption of sterile pustuleswith diffuse erythema; high fever andperipheral blood leukocytosis

Differential diagnosisPustular psoriasis; pustular bacterid; candi-diasis; impetigo herpetiformis; pyoderma

TherapyCessation of offending medication; pred-nisone

ReferencesRoujeau JC, Bioulac-Sage P, Bourseau C, Guil-

laume JC, Bernard P, et al. (1991) Acute general-ized exanthematous pustulosis. Analysis of 63 cases. Archives of Dermatology 127:1333–1338

Acute generalized exanthematous pustulosis

� Acute generalized exanthematous pustular dermatitis

Acute hemorrhagic edema of infancy

Synonym(s)Acute infantile hemorrhagic edema;Finkelstein's disease; Seidlmayer syn-drome; purpura en cocarde avec oedema;cockade purpura with edema; postinfec-tious cockade purpura of early childhood;acute benign cutaneous leukocytoclasticvasculitis of infancy

DefinitionCutaneous, small vessel leukocytoclasticvasculitis of young children with largerosetted, annular, or targetoid purpuriclesions

PathogenesisPreceded by respiratory tract infections,drug intake, or vaccination; presumablyimmune complex-mediated

Clinical manifestationLesions may begin as urticarial plaques;large, cockade (knot of ribbons appear-ance), annular, or targetoid purpuric


Acute miliary tuberculosis of skin 25

Aplaques, found primarily on the face, ears,and extremities; acral edema involving thedorsum of the hands and feet

Differential diagnosisUrticaria, acute febrile neutrophilic derma-tosis; erythema multiforme; Henoch-Schönlein purpura; leukemia cutis; menin-gococcemia or other bacterial septicemia;child abuse

Therapy None

ReferencesMillard T, Harris A, MacDonald D (1999) Acute

infantile hemorrhagic oedema. Journal of the American Academy of Dermatology 41(5 Pt 2): 837–839

Acute infantile hemorrhagic edema


Acute infective gangrene

� Necrotizing fasciitis

Acute intermittent porphyria

Synonym(s)AIP

DefinitionDefect in the enzyme porphobilinogen-deaminase that results in excessive accumu-lation of porphyrin precursors which pro-duce distinctive signs and symptoms

PathogenesisAccumulation of porphobilinogen andamino-levulinic acid (ALA), which resultsin neurologic damage that leads to periph-eral and autonomic neuropathies and psy-chiatric manifestations; autosomal domi-nant disease

Clinical manifestationMotor neuropathy that is more predomi-nant in the lower extremities; constipation;colicky abdominal pain; vomiting; periph-eral neuropathy; seizures; delirium; depres-sion; psychiatric symptoms; cortical blind-ness; coma

Differential diagnosisAbdominal diseases such as hernia, appen-dicitis; abscess, biliary disease, diverticuli-tis, gastritis; irritable bowel syndrome, aor-tic dissection, and intestinal obstruction;neurologic-psychiatric diseases such as psy-chosis, diabetic neuropathy, leprosy, nerveentrapment syndrome, and lead toxicity

TherapyGlucose, 400 g per day for treatment ofmild attacks; hematin 4 mg per kg per dayfor 4 days in severe attacks

ReferencesZaider E, Bickers DR (1998) Clinical laboratory

methods for diagnosis of the porphyrias. Clin-ics in Dermatology 16(2):277–293

Acute lupus erythematosus

� Lupus erythematosus, acute

Acute miliary tuberculosis of skin

� Cutaneous tuberculosis


26 Acute necrotizing gingivitis

Acute necrotizing gingivitis

Synonym(s)Acute necrotizing ulcerative gingivitis;trench mouth

DefinitionAcute infectious gingivitis

PathogenesisInfection of the gingiva with one of severalorganisms, including Prevotella interme-dia, alpha-hemolytic streptococci, Actino-myces species, or any of a number of differ-ent oral spirochetes; emotional stress,smoking, and poor nutrition possibly pre-disposing factors

Clinical manifestationFever; fetid breath; marked gingival edemaand ulceration, often with a grayish pseu-domembrane; most commonly involvingthe interdental papillae; may spread to adja-cent soft tissues of the mouth

Differential diagnosisDesquamative gingivitis; pemphigus vul-garis; medication toxicity (cancer chemo-therapeutic agents, etc.); aphtous stomati-tis; Behçet’s syndrome; noma

TherapyPenicillin VK�; penicillin-allergic patients:erythromycin; topical therapy: chlorhexi-dine 0.12% oral rinse used for 30 secondstwice daily; lidocaine viscous 2% applied 2–4 times daily as needed

ReferencesFenesy KE (1998) Periodontal disease: an over-

view for physicians. Mount Sinai Journal of Medicine 65(5–6):362–369

Acute necrotizing ulcerative gingivitis

� Acute necrotizing gingivitis

Acute skin failure


Acute sun damage

� Sunburn

Acute sunburn reaction

� Sunburn

Acyclovir

Trade name(s)Zovirax

Generic availableYes

Drug classAnti-viral

Mechanism of actionDNA polymerase inhibition


Adams-Oliver syndrome 27

A

Dosage form200 mg capsule; 400 mg capsule; 800 mgcapsule; 200 mg/ml oral suspension pow-der for IV solution

Dermatologic indications and dosageSee table

Common side effectsGastrointestinal: nausea; vomitingNeurologic: headache

Serious side effectsBone marrow: suppressionGastrointestinal: hepatitisNeurologic: seizures; encephalopathy; coma

Drug interactionsAminoglycoside antibiotics; carboplatin;cidofovir; cisplatin; glyburide; metformin;mycophenolate mofetil; probenecid; neph-rotoxic agents

Contraindications/precautionsHypersensitivity to drug class or compo-nent; elderly patients or those with renalfailure may need lower dose

ReferencesBrown TJ, Vander Straten M, Tyring T (2001) An-

tiviral agents. Dermatologic Clinics 19 (1):23–34

ADAM complex

� Amniotic band syndrome

Adams-Oliver syndrome

Synonym(s)Scalp and head syndrome

DefinitionCongenital absence of scalp skin with hypo-plastic or absent distal limbs

PathogenesisUnknown; autosomal dominant inherit-ance in some cases

Clinical manifestationSolitary or multiple areas of congenitalscarring alopecia of the scalp (aplasiacutis); dilated scalp veins; distal limb hypo-plasia or aplasia

Differential diagnosisFocal dermal hypoplasia; congenitalabsence of skin; constriction from amni-otic bands; trisomy 13

Acyclovir. Dermatologic indications and dosage


Eczema herpeticum 500 mg IV daily divided into 3 doses for 5 days

15 mg per kg IV daily divided into 3 doses for 5 days

Herpes simplex virus infection, 1st episode

200 mg PO 5 times daily for 10 days 5 mg per kg IV 3 times daily for 5–10 days

Herpes simplex virus infection, prophylaxis

400 mg PO twice daily for up to 1 year


Herpes simplex virus infection, recurrent

200 mg PO 5 times daily for 7 days 5 mg per kg IV 3 times daily for 5–10 days

Herpes zoster 800 mg PO 5 times daily for 7 days 20 mg per kg PO 5 times daily for 7 days

Varicella 800 mg PO 5 times daily for 7 days 20 mg per kg PO 5 times daily for 7 days


28 Adapalene

TherapySurgical correction of scalp defect�

ReferencesBeekmans SJ, Wiebe MJ (2001) Surgical treatment

of aplasia cutis in the Adams-Oliver syndrome. Journal of Craniofacial Surgery 12(6):569–572

Adapalene

Trade name(s)Differin

Generic availableNoDrug classRetinoid receptor agonist

Mechanism of actionBinds to retinoid nuclear receptors, whichmodulate differentiation, keratinization,and inflammation

Dosage form0.1% gel, solution


Common side effectsCutaneous: burning sensation; pruritus;erythema; scaling

Serious side effectsNone

Drug interactionsNone

Adapalene. Dermatologic indications and dosage


Acanthosis nigricans Apply daily, preferably at bedtime; apply 20–30 minutes after washing and drying skin

Apply daily, preferably at bedtime; apply 20–30 minutes after washing and drying skin

Acne vulgaris Apply daily, preferably at bedtime; apply 20–30 minutes after washing and drying skin


Acrokeratoelastoidosis Apply daily, preferably at bedtime Apply daily, preferably at bedtime




Actinic keratosis Apply daily, preferably at bedtime for up to 3 months

Apply daily, preferably at bedtime for up to 3 months

Melasma Apply daily, preferably at bedtime; apply 20–30 minutes after washing and drying skin


Photoaging Apply daily, preferably at bedtime; apply 20–30 minutes after washing and drying skin


Post-inflammatory hyperpigmentation



Reactive perforating collagenosis




Addison-Schilder disease 29

AContraindications/precautionsHypersensitivity to drug class or compo-nent; caution in applying to eczematousskin

ReferencesWolf JE Jr (2002) Potential anti-inflammatory ef-

fects of topical retinoids and retinoid ana-logues. Advances in Therapy 19(3):109–118

Addison disease

� Addison’s disease

Addison disease-cerebral sclerosis syndrome

� Addison-Schilder disease

Addison’s disease

Synonym(s)Addison disease; primary adrenal insuffi-ciency; chronic adrenal insufficiency;hypoadrenalism; hypocorticism; suprarenalinsufficiency

DefinitionMetabolic disease caused by an inadequatesupply or secretion of adrenocortical hor-mones, mainly mineralocorticoids and cor-tisol

PathogenesisPrimary insufficiency caused by inadequateadrenal gland function: infections (viral,tuberculosis, histoplasmosis); autoimmuneadrenal gland destruction; malignant dis-ease Suprarenal insufficiency: occurring afterabrupt discontinuance of prolonged sys-

temic corticosteroid therapy; hypopituitar-ism

Clinical manifestationUniform skin hyperpigmentation; malaise;fatigue; dizziness; anorexia; abdominalpain; hypotension; amenorrhea

Differential diagnosisAcanthosis nigricans; malnutrition;melasma; polyglandular autoimmune dis-ease; depression; hypothyroidism

TherapyCortisone 25–300 mg PO per day�; fludro-cortisone 0.1 mg PO daily�

ReferencesDon-Wauchope AC, Toft AD (2000) Diagnosis

and management of Addison's disease. Practi-tioner 244(1614):794–799

Addison-Schilder disease

Synonym(s)Addison disease-cerebral sclerosis syndro-me; Fanconi-Prader syndrome; Schilder-Addison syndrome; Siemerling-Creutzfeldtsyndrome; adrenocortical atrophy-cerebralsclerosis syndrome, adrenoleukomyelo-pathy; adrenomyelopathy; adrenomyelo-neuropathy; melanodermic leukodystro-phy; adrenoleukodystrophy

DefinitionHeritable syndrome which combines thecharacteristics of Addison’s disease (bronzeskin disease) and cerebral sclerosis(Schilder disease)

PathogenesisX-linked inheritance; disorder of lipidmetabolism and particularly the peroxi-somes; accumulation of saturated, very longchain fatty acids (VLCFA) resulting in theprogressive dysfunction of CNS white mat-ter and the adrenal cortex


30 Adenoma hidradenoides

Clinical manifestationBronze skin color; adrenal insufficiency;extensive demyelination and sclerosis of thebrain, causing behavior disturbances anddeteriorating mental and motor abnormali-ties; neurological consequences includingblindness, deafness, hemiplegia, quadriple-gia, pseudobulbar palsy, and dementia

Differential diagnosisAddison’s disease; Schilder’s syndrome

TherapySteroid replacement – cortisone acetate 25–300 mg PO every 1–2 days�; fludrocorti-sone 0.1–0.2 mg PO per day�; dietary –VLCFA-restricted diet with Lorenzo's oil

ReferencesGartner J, Braun A, Holzinger A, et al. (1998) Clin-

ical and genetic aspects of X-linked adrenoleu-kodystrophy. Neuropediatrics 29(1) 3–13

Adenoma hidradenoides

� Hidradenoma papilliferum

Adenoma sebaceum

� Angiofibroma

Adenomatosis, erosive, of nipple

� Erosive adenomatosis of the nipple

Adiponecrosis subcutanea

� Rothman-Makai syndrome

Adiposis dolorosa

� Dercum’s disease

Adrenocortical atrophy-cerebral sclerosis syndrome


Adrenoleukodystrophy


Adrenoleukomyelopathy


Adrenomyeloneuropathy


Adrenomyelopathy


African river blindness

� Filariasis


AHA revitalizing cream 31

AAfrican trypanosomiasis

Synonym(s)Sleeping sickness; human African trypano-somiasis; HAT

DefinitionInfectious parasitic disease carried by tsetseflies from the Trypanosoma brucei family,characterized by inflammation of the brainand the meninges

PathogenesisHumans infected following a tsetse fly bite;reservoir for infection in Africa; trypano-somes developing at skin innoculation siteand then invading the blood stream

Clinical manifestationEarly disease: hot, red, tender nodule atinnoculation site; regional lymphadenopa-thy.Second phase of disease: edema of theextremities and face; transient urticarial orhemorrhagic eruption; behavioral changes,alerations in sleep patterns; extrapyrami-dal neurologic signs; coma

Differential diagnosisMalaria; HIV disease; borreliosis; brucello-sis; typhoid fever; tuberculosis; bacterial,fungal, or viral meningitis

TherapyEarly disease: Suramin 100–200 mg IV testdose, then 1 g IV on days 1, 3, 7, 14�; eflorni-thine 400 mg per kg per day IV 4 timesdaily for 14 days�

Neurologic (late stage) disease: melarso-prol 2–3.6 mg per kg per day IV for 3 days;after 1 week, 3.6 mg per kg per day for 3days; after 10–21 days, repeat cycle; eflorni-thine 400 mg per kg per day IV 4 timesdaily for 14 days

ReferencesCenters for Disease Control and Prevention

Trypanosomiasis Fact Sheet. CDC May, 2000

Aggressive digital papillary adenoma

Synonym(s)Digital papillary adenoma

DefinitionBenign but locally aggressive tumor of thedigits

PathogenesisDerived from secretory eccrine sweat glandepithelium

Clinical manifestationSlowly enlarging papule or nodule on oneof the digits; occasionally eroding andbleeding; malignant variant (aggressive dig-ital papillary adenocarcinoma) having simi-lar appearance, but with histologic changesof malignancy

Differential diagnosisEccrine acrospiroma; chondroid syrin-goma; papillary eccrine adenoma; aggres-sive digital papillary adenocarcinoma

TherapyWide local excision�

ReferencesSmith KJ, Skelton HG, Holland TT (1992) Recent

advances and controversies concerning adnex-al neoplasms. Dermatologic Clinics 10(1):117–160

Aggressive fibromatosis

� Desmoid tumor

AHA revitalizing cream

� Alpha hydroxy acids


32 AHA skin smoothing cream

AHA skin smoothing cream


Ainhum

Synonym(s)Dactylolysis spontanea; constricting bandsof the extremities

DefinitionAutoamputation of a digit as a result of aconstricting scar in the form of a fibrousband or groove

PathogenesisProbably related to trauma to the affecteddigit, although exact mechanism unclear

Clinical manifestationProgressive constriction at the base of thetoe (usually the 5th toe) with distal edema;toe possibly becoming rotated, distorted atthe metatarsophalangeal joint; autoamputa-tion after the band has completely con-stricted the base of the digit

Differential diagnosisPseudoainhum; leprosy; syphilis; endemicsyphilis; pityriasis rubra pilaris; morphea;congenital constricting bands of children;pachyonychia congenita

TherapyEarly stages: relaxing incision of the fibrousbandLate stages: surgical amputation

ReferencesMarsden PD (1989) Ainhum. Transactions of the

Royal Society of Tropical Medicine & Hygiene 83(6):864

AIP

� Acute intermittent porphyria

Albendazole

Trade name(s)Albenza

Generic availableNo

Drug classAnti-helminthic

Mechanism of actionMost likely works by causing degenerationof cytoplasmic microtubules of organism,with release of proteolytic and hydrolyticenzymes in cytoplasm

Dosage form200 mg tablet


Common side effectsGastrointestinal: abdominal pain, nauseaand vomiting, meningeal signsNeurologic: headache, vertigoRenal: abnormal liver function tests

Serious side effectsBone marrow: pancytopenia, granulocyto-penia

Drug interactionsCimetidine; dexamethasone; praziquantel

Contraindications/precautionsHypersensitivity to drug class or compo-nent, specifically benzimidazole class ofcompounds


Albinoidism 33

A

ReferencesHorton J (2000) Albendazole: a review of anti-

helminthic efficacy and safety in humans. Par-asitology 121 Suppl:S113–132

Albenza

� Albendazole

Albinism

� Oculocutaneous albinism

Albinism-deafness syndrome

� Ziprkowski-Margolis syndrome

Albinoidism

Synonym(s)None

DefinitionMild form of albinism where the pigmentdilution is less marked than in other forms;absence of pigment in localized areas; thepigment in the skin, hair and eyes less thannormal but not affecting the individual asseverely as the oculocutaneous or oculartypes of albinism

PathogenesisAutosomal dominant or recessive condition

Clinical manifestationAbsence of pigment in localized areas of theskin, hair, and eyes; mild photophobia;vison less than normal but not affecting theindividual as severely as the oculocutane-ous or ocular types

Differential diagnosisOculocutaneous albinism; Hermansky-Pudlak syndrome; phenylketonuria;Chediak-Higashi syndrome; histidinemia;homocystinuria; Menkes steely hair dis-ease; Tietz syndrome; Prader-Willi syn-drome; Angelman syndrome

TherapySun protection with protective clothing andsunscreens; corrective lenses for visualimpairment

ReferencesBolognia J, Pawelek JM (1988) Biology of hypopig-

mentation. Journal of the American Academy of Dermatology 19:217–255

Albendazole. Dermatologic indications and dosage


Cutaneous larva migrans

400 mg PO daily for 3 days 15 mg per kg PO twice daily for 3 days

Cysticercosis 400 mg PO twice daily; 28–day cycle followed by 14-day rest period, for 3 cycles

< 60 kg – 15 mg per kg PO twice daily; 28–day cycle followed by 14-day rest period, for 3 cycles

Filariasis 400 mg PO as single dose 15 mg per kg PO as single dose

Strongyloidosis 200 mg PO twice daily for 3 days; repeat in 2 weeks if necessary

15 mg per kg PO twice daily for 3 days; repeat in 2 weeks if necessary


34 Albright hereditary osteodystrophy

Albright hereditary osteodystrophy

� Pseudohypoparathyroidism

Albright syndrome

� McCune-Albright syndrome

Albright-Sternberg-McCune syndrome


Albright’s syndrome


Alcaptonuria

Synonym(s)Alkaptonuria; ochronosis; homogentisicacid oxidase deficiency

DefinitionHomogentisic acid oxidase deficiencywhich results in a buildup of polymerizedphenols in skin and internal organs

PathogenesisAutosomal recessive inheritance; disorderof tyrosine (an amino acid) metabolismresulting from a defect in the enzyme

homogentisic acid oxidase; homogentisicacid oxidase deficiency leading to increasedtissue levels of homogentisic acid, whichpolymerizes non-enzymatically; deficientcollagen formation because of competitiveinhibition by homogentisic acid for ascor-bic acid

Clinical manifestationSlate blue or gray discoloration in the scle-rae and ear cartilage; diminished jointmobility; ankylosis; aortic or mitral valvuli-tis

Differential diagnosisAortic stenosis; rheumatoid arthritis, oste-oarthritis; mitral stenosis; darkened urine:acute intermittent porphyria; myoglobinu-ria; hemoglobinuria; blue discoloration:argyria; medication reaction (minocycline,amiodarone, etc); acquired ochronosis fromhydroquinone

TherapyVitamin C, up to 1 g per day PO

� Ochronosis

ReferencesLubics A, Schneider I, Sebok B, Havass Z (2000)

Extensive bluish gray skin pigmentation and severe arthropathy: endogenous ochronosis (alkaptonuria). Archives of Dermatology 136(4):548–549

Aldrich syndrome

� Wiskott-Aldrich syndrome

Aleppo oil

� Leishmaniasis, cutaneous


Alginates 35

AAlezzandrini syndrome

� Alezzandrini’s syndrome

Alezzandrini’s syndrome

Synonym(s)Alezzandrini syndrome

DefinitionDisorder consisting of unilateral tapetoreti-nal degeneration, ipsilateral appearance offacial vitiligo and poliosis, occurring inadolescents and young adults

PathogenesisUnknown

Clinical manifestationUnilateral tapetoretinal degeneration; ipsi-lateral appearance of facial vitiligo-like pig-mentaton; poliosis; occasional ipsilateralperceptual deafness; stable course withoutspontaneous re-pigmentation

Differential diagnosisPiebaldism; Waardenburg syndrome; vitil-igo; Vogt-Koyanagi-Harada syndrome

TherapyNo specific therapy

ReferencesHoffman MD, Dudley C (1992) Suspected Alezza-

ndrini's syndrome in a diabetic patient with unilateral retinal detachment and ipsilateral vi-

tiligo and poliosis. Journal of the American Academy of Dermatology 26(3 Pt 2):496–497

Alginates

Trade name(s)Kaltostat; Sorbsan; Algosteril

Generic availableNo

Drug classSynthetic dressing

Mechanism of actionAbsorbant; hemostatic

Dosage formSheet


Common side effectsPain when removed



Contraindications/precautionsNone

ReferencesThomas S (2000) Alginate dressings in surgery

and wound management – Part 1. Journal of Wound Care 9(2):56–60

Alginates. Dermatologic indications and dosage


Skin ulceration Apply directly onto ulcer bed; change when saturated with fluid

Apply directly onto ulcer bed; change when saturated with fluid


36 Algosteril

Algosteril

� Alginates

Alkaptonuria

� Alcaptonuria� Ochronosis

Allergic angiitis

� Leukocytoclastic vasculitis

Allergic angiitis and granulomatosis

� Churg-Strauss syndrome

Allergic cutaneous vasculitis


Allergic granulomatosis


Allylamine

Synonym(s)None

DefinitionChemical which inhibits squalene epoxi-dase, an enzyme in the pathway that leadsto synthesis of ergosterol, a component ofthe dermatophyte cell wall

ReferencesReitberg D (2001) Pharmacokinetics of topical

antifungal formulations. Cutis 67(5 Suppl):39–40

Alopecia

DefinitionLoss of hair, partial or complete

ReferencesHogan DJ, Chamberlain M (2000) Male pattern

baldness. Southern Medical Journal 93(7):657–662

Alopecia areata

Synonym(s)Autoimmune alopecia

DefinitionRecurrent, non-scarring type of hair loss,most likely caused by autoimmune proc-esses

PathogenesisProbably T-cell mediated; occurs in geneti-cally predisposed individuals

Clinical manifestationNon-scarring, non-inflammatory, pat-terned alopecia; one or many round-to-ovalbald patches; exclamation point hairs (i.e.hairs tapered near proximal end) oftenpresent; most commonly occurring in thescalp, but possible in any hair-bearing area


Alpha hydroxy acids 37

ADifferential diagnosisAndrogenetic alopecia; tinea capitis; pseu-dopelade of Brocq; lichen planopilaris; telo-gen effluvium; trichotillomania; syphilis

TherapyLocalized disease: triamcinolone 2–4 mgper ml intralesional; high potency topicalcorticosteroidsWidespread disease: prednisone, anthralin;topical immunotherapy with squaric acid;photochemotherapy; cyclosporine

ReferencesMadani S, Shapiro J (2000) Alopecia areata up-

date. Journal of the American Academy of Der-matology 42(4):549–566

Alopecia mucinosa

� Follicular mucinosis

Alpha hydroxy acids

Trade name(s)Aqua Glycolic lotion; Glyderm Plus; DayCream for dry skin; MD Forte facial cream;AHA Skin Smoothing Cream; AHA Revital-izing Cream

Generic availableNo

Drug classEmollient; keratolytic (chemical exfoliant)

Mechanism of actionKeratolytic at low concentration; epidermo-lysis at high concentration

Dosage formCream, lotion; various concentration/pHcombinations


Alpha hydroxy acids. Dermatologic indications and dosage


Acne vulgaris Apply twice daily Apply twice daily

Actinic keratosis Apply twice daily Apply twice daily

Dermatoheliosis Apply twice daily Apply twice daily


Apply twice daily Apply twice daily

Ichthyosis vulgaris Apply twice daily Apply twice daily

Keratosis pilaris Apply twice daily Apply twice daily

Lamellar ichthyosis Apply twice daily Apply twice daily

Melasma Apply twice daily Apply twice daily

Refsum disease Apply twice daily Apply twice daily

Rosacea Apply twice daily Apply twice daily

Tylosis Apply twice daily Apply twice daily

Ulerythema ophyrogenes


Xerosis Apply twice daily Apply twice daily

X-linked ichthyosis Apply twice daily Apply twice daily


38 Alpha interferon

Common side effectsDermatologic: skin peeling; irritation; dys-pigmentation

Serious side effectsHerpes simplex virus infection

Drug interactionsTretinoin; adapalene

Contraindications/precautionsHypersensitivity to drug class or compo-nent

ReferencesGlaser DA, Rogers C (2001) Topical and systemic

therapies for the aging face. Facial Plastic Sur-gery Clinics of North America 9(2):189–196

Alpha interferon

� Interferon-αααα

Alpha-2a interferon


Alpha-2b interferon


Alstrom’s syndrome

Synonym(s)None

DefinitionAutosomal recessive disorder with insulinresistance, diabetes mellitus, obesity, cone-rod dystrophy, and infantile cardiomyopa-thy

PathogenesisUnknown defect; autosomal recessiveinheritance

Clinical manifestationAcanthosis nigricans; retinitis pigmentosa;cardiomyopathy; deafness; obesity; diabe-tes mellitus; nephropathy; normal intelli-gence

Differential diagnosisBardet-Biedl syndrome; cone-rod dystro-phy; achromatopsia; Leber's congenitalamaurosis

TherapyTreatment of insulin resistance and diabe-tes mellitus

ReferencesRussell-Eggitt IM, Clayton PT, Coffey R, Kriss A,

Taylor DS, Taylor JF (1998) Alstrom syndrome. Report of 22 cases and literature review. Oph-thalmology 105(7):1274–1280

Aluminium chloride

Trade name(s)Drysol; Xerac-AC; Hypercare; Certain-Dri

Generic availableNo

Drug classAntiperspirant; anti-infective

Mechanism of actionReversible inhibition of eccrine gland secre-tion


Aluminium chlorohydrate 39

A

Dosage form6.25%–20% solution


Common side effectsCutaneous: stinging; burning; pruritus;skin irritation; contact dermatitis (rare)




ReferencesBenohanian A (2001) Antiperspirants and deo-

dorants. Clinics in Dermatology 19(4):398–405

Aluminium chloride hexahydrate

� Aluminium chloride

Aluminium chlorohydrate

Trade name(s)Ostiderm; Arrid XX; Right Guard Sport;Secret Antiperspirant; Dove Aerosol; SureAntiperspirant

Generic availableNo

Drug classAntiperspirant; anti-infective

Mechanism of actionReversible inhibition of eccrine gland secre-tion

Dosage formLotion, cream, roll-on


Common side effectsCutaneous: stinging; burning; pruritus; irri-tation


Aluminium chloride. Dermatologic indications and dosage


Acne, in cases where sweating is an aggravating factor

Apply daily Apply daily

Auriculotemporal syndrome

Apply daily until maximum effect is achieved; then apply 2–4 times weekly


Hyperhidrosis Apply daily until maximum effect is achieved; then apply 2–4 times weekly


Interdigital maceration




40 Aluminium granuloma



ReferencesBenohanian A (2001) Antiperspirants and deo-

dorants. Clinics in Dermatology 19(4):398–405

Aluminium granuloma

� Aluminium hypersensitivity granu-loma

Aluminium hypersensitivity granuloma

Synonym(s)Aluminium granuloma

DefinitionSubcutaneous granuloma formed as a reac-tion to aluminium-containing injectablematerial

PathogenesisFine particles of aluminium, producinglocal immunologic reaction

Clinical manifestationPruritic and tender subcutaneous nodules,appearing 2–9 months after injection ofvaccine containing aluminium as adsorb-ing agent

Differential diagnosisLupus profundus; polyarteritis nodosa; sub-cutaneous fungal infection

TherapySurgical excision�

ReferencesFawcett HA, Smith NP (1984) Injection-site gran-

uloma due to aluminium. Archives of Derma-tology 120(10):1318–1322

Alveolar soft part sarcoma

Synonym(s)Malignant organoid granular cell myoblas-toma; malignant nonchromaffin paragan-glioma

DefinitionMalignant soft tissue tumor that has anunclear origin, but distinctive histologicfindings

PathogenesisIncreased risk with Li-Fraumeni syndromeand neurofibromatosis; some associationwith Epstein Barr virus infection

Clinical manifestationSlow-growing soft tissue mass, usually inadolescents and young adults; may be pul-sitile with bruit

Differential diagnosisMetastatic disease, particularly melanomaand renal cell carcinoma; granular celltumor; leiomyosarcoma; rhabdomyosar-coma

Aluminium chlorohydrate. Dermatologic indications and dosage


Hyperhidrosis Apply daily Apply daily


Amcinonide 41

ATherapySurgical excision�

ReferencesNakashima Y, Kotoura Y, Kasakura K, Yamamuro

T, Amitani R, Ohdera K (1993) Alveolar soft-part sarcoma. A report of ten cases. Clinical Or-thopedics 294:259–266

Amalgam tattoo

Synonym(s)None

DefinitionImplantation of dental amalgam materialsinto mildly injured or inflamed mucosal tis-sues

PathogenesisImplantation of pigmented material, whichfails to evoke an inflammatory response byhost

Clinical manifestationPainless, blue/gray/black macule with nosurrounding erythematous reaction; mostfrequently found on the gingival or alveolarmucosa

Differential diagnosisNevus; melanoma; mucosal melanosis;hemangioma; venous lake; Peutz-Jegherssyndrome; medication reaction; hemochro-matosis; heavy metal intoxication

TherapyQ-switched ruby laser; excisional biopsyperformed to rule out melanoma oranother pigmented lesion

ReferencesSeward GR (1998) Amalgam tattoo. British Dental

Journal 184(10):470–471

Ambras syndrome

Synonym(s)Hypertrichosis universalis congenita,Ambras type

DefinitionCongenital hypertrichosis lanuginosa of adistinct pattern with facial dysmorphism

PathogenesisGenetic abnormality on chromosome 8 insome patients

Clinical manifestationEntire body covered with fine long hair,which spares only the palms, soles, andgenitalia; shawl-like pattern of excess hairgrowth over the shoulders; hair of the exter-nal auditory canal is long and thick; dentalabnormalities; facial dysmorphism

Differential diagnosisFamilial hypertrichosis (normal variant);hypothyroidism; anorexia nervosa; porphy-ria; mucopolysaccharidoses; GM1 gangliosi-dosis; medication-induced

TherapyLaser hair removal; depilatory cream withor without eflornithine 13.9% cream

ReferencesBaumeister FAM, Egger J, Schildhauer MT, Sten-

gel-Rutkowski S (1993) Ambras syndrome: de-lineation of a unique hypertrichosis universalis congenita and association with a balanced pericentric inversion. Clinical Genetics 44:121–128

Amcinonide

� Corticosteroids, topical, high potency


42 Amebiasis

Amebiasis

Synonym(s)Amebic dysentery; intestinal amebiasis

DefinitionProtozoal infection caused by the parasiteEntamoeba histolytica

PathogenesisAfter colonization of the colonic mucosa,the trophozoite invades intestinal mucosa,thereby gaining access to the circulation,resulting in involvement of the liver, lung,and other sites; genetic susceptibility; fac-tors such as malnutrition, sex, age, andimmunocompetence possibly alter thecourse of the infection

Clinical manifestationPapules or nodules, which may ulcerate;anogenital location most common cutane-ous site; widely disseminated lesions some-times occur in immunocompromisedpatients

Differential diagnosisInflammatory bowel disease; pyodermagangrenosum; syphilis; leishmaniasis;chancroid; anogenital carcinoma

TherapyMetronidazole 750 mg PO three times dailyfor 10 days; iodoquinol 650 mg PO threetimes daily for 20 days

ReferencesTorno MS Jr, Babapour R, Gurevitch A, Witt MD

(2000) Cutaneous acanthamoebiasis in AIDS. Journal of the American Academy of Dermatol-ogy 42(2 Pt 2): 351–354

Amebic dysentery

� Amebiasis

American trypanosomiasis

Synonym(s)Chagas disease; South American trypanoso-miasis; New World trypanosomiasis

DefinitionInfection due to the flagellate protozoaTrypanosoma cruzi, which is transmitted bythe feces of blood-sucking insect vectors(reduvid bugs)

PathogenesisEpimastigotes invading the skin at bitewound or abrasion; organisms inducing aninflammatory response, cellular lesions, andfibrosis

Clinical manifestationRed, painful papule or nodule at inocula-tion site, which sometimes ulcerates (chag-oma); conjunctivitis and periorbital edemaif the bite is near the eye (Romaña’s sign);regional lymphadenopathySystemic signs and symptoms 4–12 daysafter inoculation: fever, headache, malaise,arthralgias, and generalized lymphadenop-athyChronic phase: congestive heart failure,achalasia, megaesophagus, megacolon

Differential diagnosisLeishmaniasis; South American blastomy-cosis; atypical mycobacterial infection;inoculation deep fungal infection; cutane-ous tuberculosis; pyoderma

TherapyBenznidazole 5 mg per kg per day POdivided 2–3 times per day for 60 days

ReferencesPrata A (1994) Chagas' disease. Infectious Disease

Clinics of North America 8(1):61–76


Amniotic band syndrome 43

A

Amitriptyline

Trade name(s)Elavil; Endep


Drug classTricyclic antidepressant

Mechanism of actionInteraction with multiple neurotransmittersites, including those of norepinephrineand serotonin

Dosage form10 mg; 25 mg; 50 mg; 75 mg; 100 mg; 150 mgtablet


Common side effectsCardiovascular: tachycardiaCutaneous: dry mouthGastrointestinal: increased appetite, consti-pationGenitourinary: urinary retentionNeurologic: confusion, dizziness

Serious side effectsBone marrow: suppressionNeurologic: seizures, cerebrovascular acci-dent

Drug interactionsAcetaminophen/opiate combination drugs;alpha 2 agonists; amphetamines; antiar-rhythmics; anticholinergics; other anti-depressants; sedating antihistamines

Contraindications/precautionsHypersensitivity to drug class or compo-nent; status immediately post myocardialinfarction; MAO inhibitor use within 14days

ReferencesTennyson H; Levine N (2001) Neurotropic and

psychotropic drugs in dermatology. Dermato-logic Clinics 19(1):179–197

Amniotic band syndrome

Synonym(s)Amniotic constricting band; ADAM com-plex (amniotic deformity, adhesion, mutila-tion); Skeeter's syndrome; terminal trans-verse defects of arm; Streeter’s dysplasia

DefinitionDefects caused in the limbs or digits byentrapment in fibrous amniotic bands whilein utero

PathogenesisFibrous sticky tissues (bands) of the pla-centa occurring after rupture, entanglingthe baby, and causing limb anomalies andamputations

Amitriptyline. Dermatologic indications and dosage


Burning mouth syndrome

10–25 mg PO daily; increase by 10–15 mg every 2–3 days up to 75 mg daily

Not indicated

Post-herpetic neuralgia

10–25 mg PO daily; increase by 10–15 mg every 2–3 days up to 75 mg daily

Start at 0.1 mg/kg PO daily; increase over 2–3 weeks to 0.5 mg/kg daily


44 Amniotic constricting band

Clinical manifestationBand or indentation around an upper orlower limb or digit; amputation of a digit;facial cleft if band is across the face; abdom-inal or chest wall defect if band is locatedthere

Differential diagnosisGenetically-induced congenital anomalies

TherapySurgical correction

ReferencesWalter JH Jr, Goss LR, Lazzara AT (1998) Amniotic

band syndrome. Journal of Foot & Ankle Sur-gery 37(4):325–333

Amniotic constricting band


Amoxicillin

Trade name(s)Amoxil; Trimox


Drug classPenicillins

Mechanism of actionBacterial cell wall synthesis inhibition

Dosage form250 mg; 500 mg tablets


Common side effectsBone marrow: eosinophilia

Amoxicillin. Dermatologic indications and dosage


Acute paronychia 250–500 mg PO 3 times daily for 10 days

250 mg PO 3 times daily for 10 days

Atrophoderma of Pasini and Pierini

250–500 mg PO 3 times daily for 21 days


Cutaneous anthrax 500 mg PO 3 times daily for 7–10 days (60 days in bio terrorism setting

250 mg PO 3 times daily for 7–10 days (60 days in bio terrorism setting)

Glanders 60 mg per kg daily, divided into 3 daily doses for 60–150 days

60 mg per kg daily, divided into 3 daily doses for 60–150 days

Leptospirosis 0.5–1 gm PO 3 times daily for 14–21 days

250–500 mg PO 3 times daily for 14–21 days

Lyme disease 250–500 mg PO 3 times daily for 21 days

20–50 mg per kg PO divided into 3 doses daily for 3 weeks

Melioidosis 60 mg per kg daily, divided into 3 daily doses for 60–150 days

60 mg per kg daily, divided into 3 daily doses for 60–150 days

Pyoderma 250–500 mg PO 3 times daily for 10 days


Salmonellosis 1 gm PO every 8 hours for 10–14 days

250–500 mg PO every 8 hours for 10–14 days


Amyloid 45

ACutaneous: urticaria or other vascular reac-tionGastrointestinal: nausea and vomiting,diarrhea; elevated liver enzymes

Serious side effectsBone marrow: suppressionCutaneous: Stevens-Johnson syndrome,toxic epidermal necrolysis, anaphylaxisGastrointestinal: pseudomembranous coli-tis

Drug interactionsAminoglycoside antibiotics; allopurinol;oral contraceptives; probenecid; methotrex-ate

Contraindications/precautionsHypersensitivity to drug class or compo-nent; caution if there is a cephalosporinallergy; caution if patient is suspected ofhaving EB virus infection; caution withimpaired renal function

ReferencesSteere AC (1997) Diagnosis and treatment of Lyme

arthritis. Medical Clinics of North America 81(1):179–194

Amoxil

� Amoxicillin

Ampicillin

Trade name(s)Omnipen; Principen


Drug classPenicillins

Mechanism of actionBacterial cell wall synthesis inhibition

Dosage form250 mg, 500 mg tablets; 125, 250 mg/5 mlsuspension


Common side effectsBone marrow: eosinophiliaCutaneous: urticaria or other vascular reac-tionGastrointestinal: nausea and vomiting,diarrhea, elevated liver enzymes

Serious side effectsBone marrow: suppressionCutaneous: Stevens-Johnson syndrome,toxic epidermal necrolysis, anaphylaxisGastrointestinal: pseudomembranous coli-tis

Drug interactionsAminoglycoside antibiotics; allopurinol;oral contraceptives; probenecid; methotrex-ate

Contraindications/precautionsHypersensitivity to drug class or compo-nent; caution if there is a cephalosporinallergy; caution if patient is suspected ofhaving EB virus infection; caution withimpaired renal function

ReferencesSadick N (2000) Systemic antibiotic agents. Der-

matologic Clinics 19(1):1–21

Amsterdam syndrome

� Cornelia de Lange syndrome

Amyloid

� Amyloidosis


46 Amyloidosis

Amyloidosis

Synonym(s)Amyloid

DefinitionDisorder in which insoluble protein fibersare deposited in tissues, causing impairedfunction

ReferencesGertz MA, Lacy MQ, Dispenzieri A (1999) Amy-

loidosis: recognition, confirmation, prognosis, and therapy. Mayo Clinic Proceedings 74(5):490–494

Amyopathic dermatomyositis

� Dermatomyositis

Anagen effluvium

Synonym(s)Chemotherapy-induced alopecia

DefinitionHair loss after any insult to the hair folliclethat impairs its mitotic or metabolic activ-ity

PathogenesisInhibition or arrest of cell division in thehair matrix by toxins, resulting in a thin,

weakened hair shaft that is susceptible tofracture with minimal trauma or to com-plete failure of hair formation

Clinical manifestationDiffuse, non-inflammatory, non-scarringalopecia, which begins 7–14 days afterchemotherapy pulse, especially with doxo-rubicin, the nitrosoureas, and cyclophos-phamide

Differential diagnosisTelogen effluvium; androgenetic alopecia;alopecia mucinosa; traction alopecia; looseanagen syndrome; follicular degenerationsyndrome; malnutrition; thyroid disease;diabetes mellitus; Sézary syndrome

TherapyMinoxidil 5% solution

ReferencesDuvic M, Lemak NA, Valero V, et al. (1996) A ran-

domized trial of minoxidil in chemotherapy-induced alopecia. Journal of the American Academy of Dermatology 35(1):74–78

Anal itching

� Pruritus ani

Anaphylactic reaction

� Anaphylaxis

Ampicillin. Dermatologic indications and dosage


Disseminated gonococcal infection

1 gm PO every 6 hours for 7–10 days 500 mg PO every 6 hours for 7–10 days

Pyoderma 250–500 mg PO 4 times daily for 10 days

< 7 years old – 125 mg PO 4 times daily for 10 days


Androgenetic alopecia 47

AAnaphylactoid purpura

� Henoch-Schönlein purpura

Anaphylactoid reaction

� Anaphylaxis

Anaphylaxis

Synonym(s)Systemic allergic reaction; anaphylacticreaction; anaphylactoid reaction

DefinitionAcute systemic reaction caused by therelease of mediators from mast cells andbasophils and involving more than oneorgan system

PathogenesisType I hypersensitivity reaction with mastcell degranulation mediated by antigenbinding of specific immunoglobulin E (IgE);released mediators causing smooth musclespasm in the bronchi and gastrointestinaltract, vasodilation, increased vascular per-meability, and stimulation of sensory nerveendings

Clinical manifestationCutaneous manifestations: urticaria, angio-edema, conjunctival pruritusSystemic manifestations: tightness, short-ness of breath, chest pain, palpitations, syn-cope, nausea, vomiting, diarrhea

Differential diagnosisVasovagal syndrome; mastocytosis; carci-noid syndrome; pheochromocytoma; panicattack

TherapySecuring of airway; removal of antigenicsource, if possible (e.g. bee stinger); tourni-quet applied to the extremity with the anti-gen source, if known; epinephrine 0.3–0.5 mL (0.3–0.5 mg) of 1:1000 solution viasubcutaneous or intramuscular route,repeated as needed; diphenhydramine 10–50 mg via either intravenous or intramus-cular route every 4 hours as needed; raniti-dine 50 mg via either intravenous or intra-muscular route every 6–8 hours

ReferencesRing J, Behrendt H (1999) Anaphylaxis and ana-

phylactoid reactions. Classification and patho-physiology. Clinical Reviews in Allergy & Immunology 17(4):387–399

Androgenetic alopecia

Synonym(s)Common baldness; familial baldness;hereditary baldness; male pattern baldness;female pattern baldness; pattern baldness

DefinitionPhysiologic process in genetically predis-posed individuals who develop a patternedscalp hair loss characterized by progressiveminiaturization of the follicles and progres-sive conversion from terminal hair to vellushair

PathogenesisGenetically determined characteristics ofhair follicles in the scalp, causing the folli-cles to be more likely to miniaturize in thepresence of androgens, particularly dihy-drotestosterone

Clinical manifestationProgressive, patterned, non-inflammatory,and non-scarring alopecia of the scalp


48 Anetoderma

Differential diagnosisTelogen effluvium; alopecia areata; anageneffluvium; virilizing disorders in women;thyroid disease; iron deficiency

TherapyMinoxidil; finasteride (men only)

ReferencesSinclair RD, Dawber RP (2001) Androgenetic alo-

pecia in men and women. Clinics in Dermatol-ogy 19(2):167–178

Anetoderma

Synonym(s)None

DefinitionLocalized laxity of the skin with herniationor out-pouching, resulting from abnormalelastic tissues

ReferencesKarrer S, Szeimies RM, Stolz W, Landthaler M

(1996) Primary anetoderma in children: report of two cases and literature review. Pediatric Dermatology 13(5):382–385

Angel wing deformity

DefinitionFocal destruction of nail matrix in lichenplanus, producing central scarred area(pterygium) and peripheral area of pre-served nail, simulating angel wings

ReferencesMirza B, Ashton R (2000) Recognising common

nail conditions: a guide. Practitioner 244(1615):873–874, 876–878, 882–883

Angel's kiss

� Salmon patch

Angelman syndrome

Synonym(s)None

DefinitionDevelopmental syndrome of mental retar-dation, abnormal behavior, and hypopig-mentation

PathogenesisChromosomal and molecular changes ofthe proximal region of chromosome 15

Clinical manifestationSmall stature; developmental delay; nospeech; abnormal shape of head; protrud-ing, large tongue; behavioral abnormali-ties; skin pigment dilution of the skin andeyes

Differential diagnosisPrader-Willi syndrome; oculocutaneousalbinism

TherapySun protection

ReferencesLaan LA, Haeringen A, Brouwer OF (1999) Angel-

man syndrome: a review of clinical and genetic aspects. Clinical Neurology & Neurosurgery 101(3):161–170

Angio-osteohypertrophy

� Klippel-Trenaunay-Weber Syndrome


Angioendotheliomatosis 49

AAngioblastoma

� Tufted angioma

Angiocentric lymphoproliferative lesion

� Lymphomatoid granulomatosis

Angiodermité de Favre et Chaix


Angioedema

DefinitionAsymptomatic, non-pitting, and well-cir-cumscribed areas of edema due toincreased vascular permeability

ReferencesKaplan AP (2002) Clinical practice. Chronic urti-

caria and angioedema. New England Journal of Medicine 346(3):175–179

Angioendotheliomatosis

Synonym(s)Intravascular lymphomatosis; malignantangioendotheliomatosis; angioendothelio-matosis proliferans systematica; proliferat-

ing endotheliosis; angioendo-theliomatosisproliferans; intravascular endothelioma;reactive inflammatory systematizedangioendotheliomatosis; reactive angioen-dotheliomatosis; proliferating systematizedendotheliosis

DefinitionBenign reactive form: proliferation of cellsexpressing endothelial cell markersMalignant form: angiotropic B cell lym-phoma

PathogenesisBenign reactive form: occlusion of vascularlumina a common feature; associated withsystemic infections, paraproteinemias,monoclonal gammopathies, iatrogenic arte-riovenous fistulas, antiphospholipid syn-dromeMalignant form: vascular occlusion fromsludging of the circulating malignant lym-phoid cells

Clinical manifestationIndurated, red, or violaceous papules form-ing plaques or nodules which may ulcerate;located over the abdominal region, lowerextremities, trunk, arms, and face

Differential diagnosisAngiosarcoma; acroangiodermatitis; tuftedangioma; peripheral T-cell lymphoma; cry-oglobulinemia; perniosis; vasculitis; bacil-lary angiomatosis; Kaposi’s sarcoma;endovascular papillary angioendotheliomaof childhood; angioimmunoblastic lym-phadenopathy; angiolymphoid hyperplasia

TherapyBenign reactive form: no effective therapyMalignant form: treatment for systemiclymphoma

ReferencesBerger TG. Dawson NA. Angioendotheliomatosis.

Journal of the American Academy of Dermatol-ogy 18(2 Pt 2):407–412, 1988


50 Angioendotheliomatosis proliferans

Angioendotheliomatosis proliferans

� Angioendotheliomatosis

Angioendotheliomatosis proliferans systematica


Angiofibroma

Synonym(s)Adenoma sebaceum; fibrous papule of thenose and face; pearly penile papules; oralfibroma

DefinitionHistologic entity characterized by dermalfibrovascular proliferation

PathogenesisUnknown; a cutaneus manifestation oftuberous sclerosis, where it represents ahamartoma

Clinical manifestationSolitary or multiple firm, discrete, flesh-colored-to-telangiectatic papules

Differential diagnosisFlat warts; molluscum contagiosum; follicu-litis; nevus; basal cell carcinoma; cherryangioma; sarcoidosis; granuloma annulare;acne vulgaris; rosacea; appendageal tumors

TherapyShave removal; destruction by electrodesic-cation and curettage; laser vaporization;dermabrasion

ReferencesMorelli JG (1998) Use of lasers in pediatric derma-

tology. Dermatologic Clinics 16(3):489–495

Angioid streak

DefinitionLinear, gray or dark red lines with irregularserrated edges lying beneath normal reti-nal blood vessels, resulting from pathologi-cal changes at the level of the Bruch mem-brane

ReferencesGurwood AS, Mastrangelo DL (1997) Under-

standing angioid streaks. Journal of the Amer-ican Optometric Association 68(5):309–324

Angiokeratoma circumscriptum

Synonym(s)Corporis circumscriptum naeviforme;angiokeratoma corporis neviform

DefinitionVascular ectasia involving the papillary der-mis, producing unilateral hyperkeratoticplaques

PathogenesisUnknown mechanism for development,possibly involving altered hemodynamicswhich produce telangiectatic vessels of thedermis with an overlying reactive epider-mal hyperkeratosis

Clinical manifestationHyperkeratotic, verrucous, dark red-to-purple, slightly compressible papules orplaques, sometimes in a linear distribution

Differential diagnosisAngioma corporis diffusum; cherry heman-gioma; elastosis perforans serpiginosa; blue


Angiokeratoma of Mibelli 51

Arubber bleb nevus; angioma serpiginosum;lymphangioma circumscriptum; verrucoushemangioma

TherapySurgical treatment for cosmesis: surgicalexcision; flash pump dye or diode laserablation; destruction by electrodesiccationand curettage; liquid nitrogen cryotherapy

ReferencesSchiller PI, Itin PH (1996) Angiokeratomas: an

update. Dermatology 193(4):275–282

Angiokeratoma corporis diffusum

Synonym(s)Fabry disease; Fabry-Anderson disease;Fabry syndrome

DefinitionX-linked, inherited disorder caused by adeficiency of the lysosomal enzyme alpha-galactosidase

PathogenesisDefect in the activity of lysosomal alpha-galactosidase, resulting in the storage oftwo neutral glycosphingolipids, trihexosyl-ceramide and digalactosylceramide; accu-mulation in many types of cells, includingvascular endothelium

Clinical manifestationMultiple, verrucous, red-to-violaceouspapules, with a predilection for the scro-tum, penis, lower back, thighs, hips, but-tocks, and lips; lesions typically sparing theface, scalp, and ears; progressive neuro-logic, heart, and kidney disease

Differential diagnosisAngiokeratoma of the scrotum; adult typebeta-galactosidase deficiency; aspartylglu-cosaminuria; adult onset variant of alpha-N-acetylgalactosaminidase deficiency;fucosidosis; sialidosis

TherapyNo specific therapy for underlying defect;destruction of individual lesions with elec-trodesiccation and curettage or CO2 laservaporization

ReferencesPastores GM, Lien YH (2002) Biochemical and

molecular genetic basis of Fabry disease. Jour-nal of the American Society of Nephrology 13 Suppl 2:S130–133

Angiokeratoma corporis neviform

� Angiokeratoma circumscriptum

Angiokeratoma of Fordyce

� Angiokeratoma of scrotum

Angiokeratoma of Mibelli

Synonym(s)Naevus a pernione

DefinitionVascular ectasia involving the papillary der-mis and producing a hyperkeratotic plaque

PathogenesisUnknown

Clinical manifestationFriable, verrucous, blue-red or gray papule,sometimes with a central crust, occurringin childhood; may involute after minortrauma; associated with acrocyanosis andchilblains

Differential diagnosisWart; hemangioma; lymphangioma; pyo-genic granuloma; amelanotic melanoma;


52 Angiokeratoma of scrotum

seborrheic keratosis; blue rubber blebnevus

TherapyDestruction by either liquid nitrogen cryo-therapy, electrodessication and curettage,or laser ablation



Angiokeratoma of scrotum

Synonym(s)Angiokeratoma of Fordyce; Fordyceangiokeratoma; angiokeratoma scroti;angiokeratoma of the vulva; angiokeratomavulvae

DefinitionVascular ectasia involving the papillary der-mis and producing unilateral hyperkera-totic papules of the scrotum or vulva

PathogenesisIncreased venous pressure possible causa-tive factor

Clinical manifestationSolitary or multiple friable 2–3 mm red-to-blue papules on the scrotum or labia majora

Differential diagnosisAngiokeratoma corporis diffusum; genitalwart; melanoma; pyogenic granuloma; lym-phangioma; seborrheic keratosis; blue rub-ber bleb nevus

TherapyDestruction by either liquid nitrogen cryo-therapy, electrodesiccation and curettage,or laser ablation



Angiokeratoma of vulva


Angiokeratoma scroti


Angiokeratoma vulvae


Angiolipoma

Synonym(s)None

DefinitionBenign tumor of subcutaneous fat with anexcessive degree of vascular proliferation

PathogenesisUnknown

Clinical manifestationPainful subcutaneous papule or nodule

Differential diagnosisLipoma; eccrine spiradenoma; leiomyoma;neuroma; glomus tumor; Dercum’s disease;hibernoma; liposarcoma

TherapySurgical excision; liposuction

ReferencesAlvi A, Garner C, Thomas W (1998) Angiolipoma

of the head and neck. Journal of Otolaryngolo-gy 27(2):100–103


Angry back syndrome 53

AAngiolupoid sarcoid

� Sarcoidosis

Angiolymphoid hyperplasia with eosinophilia

Synonym(s)Epithelioid hemangioma; histiocytoidhemangioma; pseudopyogenic granuloma;papular angioplasia; inflammatory angiom-atous nodules

DefinitionBenign vascular tumor of the head andneck region, often associated with periph-eral eosinophilia

PathogenesisUnclear, but possibly a reactive processrather than a true neoplasm

Clinical manifestationEnlarging, dome-shaped, red-to-brown,solitary or multiple papules or nodules,usually in the head and neck area; may beassociated with pain or pruritus; periph-eral eosinophilia in 20% of cases

Differential diagnosisGranuloma faciale; hemangioma; lym-phoma; pseudolymphoma; Kaposi’s sar-coma; angiosarcoma; insect bite reaction;pyogenic granuloma; amelanotic melanoma

TherapySurgical excision, to include the arterial andvenous segments at the base of the lesion�;superificial radiation; intralesional triamci-nolone

ReferencesMariatos G, Gorgoulis VG, Laskaris G, Kittas C

(1999) Epithelioid hemangioma (angiolym-phoid hyperplasia with eosinophilia) in the

oral mucosa. A case report and review of the lit-erature. Oral Oncology 35(4):435–438

Angioma

� Hemangioma

Angioma pigmentosum et atrophicum

� Xeroderma pigmentosum

Angiosarcoma

Synonym(s)Malignant angioma; malignant endothe-lioma

DefinitionMalignant neoplasm derived from bloodvessels and characterized by rapidly prolif-erating, extensively infiltrating, anaplasticcells

ReferencesBrown MD (2000) Recognition and management

of unusual cutaneous tumors. Dermatologic Clinics 18(3):543–552

Angry back syndrome

DefinitionCondition whereby a strongly positivepatch test causes increase in the number ofpositive patch tests at nearby sites

ReferencesFisher AA (1996) The evolution of the terminolo-

gy of “crazy” or “angry” back syndrome in


54 Anguillulosis

patch testing procedures: Part I. Cutis 58(6):389–390

Anguillulosis

� Strongyloidosis

Angular cheilitis

Synonym(s)None

DefinitionInflammation of the skin and mucousmembranes of the angles of the mouth

PathogenesisFour factors implicated: 1) infections;2) mechanical factors; 3) nutritional defi-ciencies; 4) pre-existent skin diseases

Clinical manifestationTriangular area of erythema, edema, scale,and fissuring at the corners of the mouth;recurrent exudation and crusting; candida acommon pathogen

Differential diagnosisCongenital syphilis; actinic cheilitis

TherapyMedical: fluconazole; azole antifungalcream; low potency topical corticosteroid;bovine collagen filler injected intrader-mally to corners of the mouth to restorecontourNon-medical: new dentures to restore facialcontour; correction of nutritional deficien-cies with multivitamins, etc.

ReferencesRogers RS 3rd, Bekic M (1997) Diseases of the lips.

Seminars in Cutaneous Medicine & Surgery 16(4):328–336

Anhidrosis

DefinitionAbnormal lack of sweat in response to heat

ReferencesLeung AK, Cho HY, Choi MC, Chan PY (1999) Hy-

pohidrosis in children. Journal of the Royal So-ciety of Health 119(2):101–107

Anhidrotic ectodermal dysplasia

Synonym(s)Hypohidrotic ectodermal dysplasia; Christ-Siemens-Touraine syndrome

DefinitionSyndrome consisting of anhidrosis or hypo-hidrosis, defective dentition, and hypotri-chosis

PathogenesisX-linked disorder, or rarely, autosomalrecessive disorder; female carriers mildlyaffected, possibly because of inactivation ofX chromosome

Clinical manifestationPyrexia secondary to inadequate sweating;abnormal facies; sparse hair; abnormalnails; skin dryness; markedly dystrophicteeth with early caries

Differential diagnosisHidrotic ectodermal dysplasia; Rapp-Hodg-kin syndrome; Rosselli-Giulienetti syn-drome; ectrodactyly ectodermal dysplasiaclefting syndrome

TherapyPrevention of overheating; regular dentalcare; emollients for dry skin


Anthralin 55

AReferencesVasan N (2000) Management of ectodermal dys-

plasia in children – an overview. Annals of the Royal Australasian College of Dental Surgeons 15:218–222

Annular

Synonym(s)None

DefinitionDescriptive term of a lesion with anadvancing, active margin with central clear-ing

ReferencesHsu S, Le EH, Khoshevis MR (2001) Differential

diagnosis of annular lesions. American Family Physician 64(2):289–296

Annular elastolytic giant-cell granuloma

� Actinic granuloma

Anogenital verrucous carcinoma

� Giant condyloma of Buschke and Löwenstein

Anogenital wart

� Condyloma acuminatum

Anonychia

DefinitionAbsence of nails from birth

ReferencesAl Hawsawi K, Al Aboud K, Alfadley A, Al Aboud

D (2002) Anonychia congenita totalis: a case re-port and review of the literature. International Journal of Dermatology 41(7):397–399

Anthralin

Trade name(s)Anthro-derm; Drithocreme; Dritho-Scalp;Micanol


Drug classAntimitotic; immunomodulator

Mechanism of actionMay work by stimulating monocyte pro-inflammatory activity and/or throughantimitotic effects that result from inhibi-tion of DNA synthesis; induces extracellu-lar free radicals

Dosage form 0.1%, 0.25%, 0.5%, 1% cream and ointment;


Common side effectsCutaneous: skin irritation; allergic contactdermatitis; erythemaMiscellaneous: discoloration of skin, hair,and nails; staining of clothing, sinks, bath-tubs, and furniture



56 Anthrax, cutaneous


Contraindications/precautionsHypersensitivity to drug class or compo-nent; avoid heating lesions on the face,intertriginous areas; use with caution oninflamed skin; to minimize discoloration,rinse the bath/shower with hot water imme-diately after washing/showering and thenuse a suitable cleanser to remove anydeposit on the surface of the bath or shower

ReferencesLebwohl M, Ali S (2001) Treatment of psoriasis.

Part 1. Topical therapy and phototherapy. Jour-nal of the American Academy of Dermatology 45(4):487–498

Anthrax, cutaneous

Synonym(s)Malignant pustule; woolsorter's disease;black bane; charbon; murrain; black blood

DefinitionSkin disease resulting from exposure to thespores of Bacillus anthracis

PathogenesisCutaneous invasion by Bacillus anthracis;may gain access through microscopic orgross breaks in the skin; may occur after

handling sick animals or contaminatedwool, hair, or animal hides

Clinical manifestation1–7 days (usually 2–5) incubation periodafter skin exposure; starts as pruriticpapule that enlarges in 24–48 hours to forman ulcer, evolves into a black eschar, andlasts for 7–14 days before separating andleaving a permanent scar; regional lym-phadenopathy, which may be present forweeks after the ulceration heals

Differential diagnosisBubonic plague; tularemia; syphilis; staphy-lococcal pyoderma; cat-scratch disease;cowpox; North American blastomycosis;sporotrichosis; atypical mycobacterialinfection; orf; milker’s nodule; leishmania-sis

TherapyPenicillin�; doxycycline; ciprofloxacin;amoxicillin

ReferencesTutrone WD, Scheinfeld NS, Weinberg JM (2002)

Cutaneous anthrax: a concise review. Cutis 69(1):27–33

Anticardiolipin antibody syndrome

� Antiphospholipid syndrome

Anthralin. Dermatologic indications and dosage


Alopecia areata Apply for 30 minutes; wash off with warm water without soap; start at low concentration (0.1–0.25%) and titrate upward as tolerated

Apply for 30 minutes; wash off with warm water without soap; start at low concentration (0.1–0.25%) and titrate upward as tolerated

Psoriasis Apply for 30 minutes; wash off with warm water without soap; start at low concentration (0.1–0.25%) and titrate upward as tolerated

Apply for 30 minutes; wash off with warm water without soap; start at low concentration (0.1–0.25%) and titrate upward as tolerated


Antihistamines, second generation 57

AAnticardiolipin syndrome


Antihistamines, first generation

Trade name(s)Generic names in parentheses:Benadryl, Dermarest, Sominex (diphenhy-dramine); Pyribenzamine (tripelen-namine); Periactin (cyproheptadine); Phen-ergan (promethazine); Chlor-Trimeton,Comtrex (chlorpheniramine); Polaramine(dexchlorpheniramine); Atarax, Vistaril(hydroxyzine); Dimetane (bromphe-niramine); Sinequan (doxepin)


Drug classAntihistamine

Mechanism of actionCompetitive inhibitor of histamine at H-1receptor site

Dosage formTablet; elixir; capsule; syrup

Dermatologic indicationsSee table

Common side effectsDermatologic: dry mouthNeurologic: ataxia, dizziness, headache, agi-tationGastrointestinal: diarrhea

Serious side effectsNeurologic: dyskinesia, seizuresRespiratory: wheezing

Drug interactionsAnticholinergics; antidepressants; antipsy-chotics; barbiturates; opiates; sedative hyp-notics

Other interactionsEthanol

Contraindications/precautionsHypersensitivity to drug class or compo-nent; pregnancy, first trimester; caution inasthmatics

ReferencesGreaves MW (2001) Antihistamines. Dermatolog-

ic Clinics 19(1):53–62

Antihistamines, second generation

Trade name(s)Generic names in parentheses:Allegra (fexofenadine); Claritin (lorata-dine); Clarinex (desloratadine); Zyrtec(cetirizine)


Drug classAntihistamine

Mechanism of actionCompetitive inhibitor of histamine at H-1receptor site

Dosage formTablet; capsule; syrup


Common side effectsCutaneous: dry mouth


58 Antihistamines, second generation

Antihistamines, first generation. Dermatologic indications and dosage


Atopic dermatitis Diphenhydramine 25–50 mg at bedtime for sedation; hydroxyzine 10–25 mg PO at bedtime for sedation.Doxepin 10–50 mg PO at bedtime for sedation

Diphenhydramine 10–25 mg at bedtime for sedation; hydroxyzine 10–25 mg PO at bedtime for sedation.Doxepin 10–25 mg PO at bedtime for sedation

Autoerythrocyte sensitization syndrome

Diphenhydramine 25–50 mg PO 4 times daily; hydroxyzine 10–25 mg PO 4 times daily

Diphenhydramine 5 mg per kg PO daily, divided into 4 doses; hydroxyzine 10–25 mg PO 4 times daily.Doxepin 10–25 mg PO at bedtime for sedation

Cercarial dermatitis Diphenhydramine 25–50 mg at bedtime for sedation; hydroxyzine 10–25 mg PO at bedtime for sedation.Doxepin 10–50 mg PO at bedtime for sedation


Dermatographism Diphenhydramine 25–50 mg PO 4 times daily; hydroxyzine 10–25 mg PO 4 times daily.Doxepin 10–50 mg PO at bedtime for sedation


Erythema infectiosum Diphenhydramine 25–50 mg at bedtime for sedation; hydroxyzine 10–25 mg PO at bedtime for sedation.Doxepin 10–50 mg PO at bedtime for sedation


Erythema multiforme Diphenhydramine 25–50 mg at bedtime for sedation; hydroxyzine 10–25 mg PO at bedtime for sedation.Doxepin 10–50 mg PO at bedtime for sedation


Insect bite reaction Diphenhydramine 25–50 mg at bedtime for sedation; hydroxyzine 10–25 mg PO at bedtime for sedation.Doxepin 10–50 mg PO at bedtime for sedation



Antihistamines, second generation 59

A

Lichen simplex chronicus



Mastocytosis Diphenhydramine 25–50 mg PO 4 times daily; hydroxyzine 10–25 mg PO 4 times daily.Doxepin 10–50 mg PO at bedtime for sedation


Pruritus Diphenhydramine 25–50 mg at bedtime for sedation; hydroxyzine 10–25 mg PO at bedtime for sedation.Doxepin 10–50 mg PO at bedtime for sedation


Seabather’s eruption Diphenhydramine 25–50 mg at bedtime for sedation; hydroxyzine 10–25 mg PO at bedtime for sedation.Doxepin 10–50 mg PO at bedtime for sedation


Serum sickness Diphenhydramine 25–50 mg PO 4 times daily; hydroxyzine 10–25 mg PO 4 times daily.Doxepin 10–50 mg PO at bedtime for sedation


Uremic pruritus Diphenhydramine 25–50 mg at bedtime for sedation; hydroxyzine 10–25 mg PO at bedtime for sedation.Doxepin 10–50 mg PO at bedtime for sedation


Urticaria Diphenhydramine 25–50 mg PO 4 times daily; hydroxyzine 10–25 mg PO 4 times daily.Doxepin 10–50 mg PO at bedtime for sedation


Antihistamines, first generation. Dermatologic indications and dosage (Continued)



60 Antiphospholipid antibody syndrome

Gastrointestinal: nausea, diarrheaNeurologic: somnolence, fatigue, dizziness,agitation, headache

Serious side effectsRespiratory: hypersensitivity reaction,bronchospasm

Drug interactionsAnticholinergics; antidepressants; antipsy-chotics; barbiturates; opiates; sedative hyp-notics


Contraindications/precautionsHypersensitivity to drug class or compo-nent; caution in patients with impairedrenal or liver function

ReferencesGreaves MW (2001) Antihistamines. Dermatolog-


Antiphospholipid antibody syndrome


Antiphospholipid syndrome

Synonym(s)Antiphospholipid antibody syndrome;anticardiolipin syndrome; anticardiolipinantibody syndrome

Xerosis with pruritus Diphenhydramine 25–50 mg at bedtime for sedation; hydroxyzine 10–25 mg PO at bedtime for sedation.Doxepin 10–50 mg PO at bedtime for sedation


Antihistamines, first generation. Dermatologic indications and dosage (Continued)


Antihistamines, second generation. Dermatologic indications and dosage


Dermatographism Fexofenadine 180 mg PO daily; loratadine 10 mg PO daily; desloratadine 5 mg PO daily; cetirizine 10 mg PO daily

Fexofenadine 30 mg PO twice daily; loratadine 5 mg PO daily; cetirizine 2.5 mg PO daily

Mastocytosis Fexofenadine 180 mg PO daily; loratadine 10 mg PO daily; desloratadine 5 mg PO daily; cetirizine 10 mg PO daily


Urticaria Fexofenadine 180 mg PO daily; loratadine 10 mg PO daily; desloratadine 5 mg PO daily; cetirizine 10 mg PO daily



Aphthous stomatitis 61

ADefinitionDisorder characterized by recurrent,venous or arterial thrombosis and/or fetallosses associated with antibodies directedagainst membrane anionic phospholipids(i.e. anticardiolipin [aCL] antibody,antiphosphatidylserine) or their associatedplasma proteins, predominantly beta-2glycoprotein I (apolipoprotein H), or evi-dence of a circulating anticoagulant

PathogenesisUnclear mechanism, possibly representinga defect in cellular apoptosis, that exposesmembrane phospholipids to the binding ofvarious coagulation proteins, which subse-quently become the target of autoantibod-ies; hypercoagulable state resulting in clini-cal signs and symptoms of disease

Clinical manifestationHistory of deep vein thrombosis (DVT),pulmonary embolism, acute ischemia, myo-cardial infarction, or CVA, often at an earlyage; frequent miscarriages or prematurebirths; livedo reticularis; superficial throm-bophlebitis; leg ulcers; painful purpura;splinter hemorrhages

Differential diagnosisEndocarditis; disseminated intravascularcoagulation; thrombotic thrombocytopenicpurpura; hypercoagulable state from othercauses such as malignancy; atheroscleroticvascular disease; multiple cholesterolemboli; systemic necrotizing vasculitis

TherapyElimination of risk factors, such as oralcontraceptives, smoking, hypertension, andhyperlipidemia; aspirin 81 mg PO per day;warfarin: 2–15 mg PO per day; enoxaparin:1 mg per kg subcutaneously twice daily

ReferencesGharavi AE (2001) Anticardiolipin syndrome: an-

tiphospholipid syndrome. Clinical Medicine 1(1):14–17

Apert’s syndrome

� Acrocephalosyndactyly

Aphthae

� Aphthous stomatitis

Aphthous stomatitis

Synonym(s)Aphthae; recurrent aphthous stomatitis;recurrent aphthous ulcers; canker sores;periadenitis mucosa necrotica recurrens

DefinitionBenign mouth lesion, presenting as a pain-ful white or yellow ulceration

PathogenesisPossible inherited predisposition; possibleimmune system dysfunction; trauma (den-tal procedures or aggressive tooth clean-ing) precipitates lesions

Clinical manifestationAphthae minor: recurrent, discrete, pain-ful, shallow ulcers measuring from 3 mm to< 1 cm occurring on the labial and buccalmucosa and the floor of the mouth; lesionsheal without scarring within 7–10 daysAphthae major: oval-shaped ulcers from 1–3 cm in diameter; multiple lesions oftenpresent simultaneously; healing takes up to6 weeks

Differential diagnosisOral cancer; contact dermatitis; erythemamultiforme; herpes simplex virus infection;hand-foot-and-mouth disease; lichen pla-nus; lupus erythematosus; pemphigus vul-garis; paraneoplastic pemphigus; Reiter


62 Aplasia cutis congenita

syndrome; syphilis; traumatic ulceration;drug reaction; Behçet’s disease; cyclic neu-tropenia

TherapyTopical therapy: Kaopectate applied to ulcer3–4 times per day; Zilactin gel applied 4–5times per day; high potency topical corti-costeroids; viscous Xylocaine applied asneeded; amlexanox 5% paste applied 4times daily; tetracycline suspension(250 mg capsule contents suspended in 5 mlof water) applied to mouth or genital ulcers4 times dailySystemic therapy (used mostly for aphthaemajor): thalidomide; prednisone; colchi-cine; azathioprine

ReferencesPorter SR, Hegarty A, Kaliakatsou F, Hodgson TA,

Scully C (2000) Recurrent aphthous stomatitis. Clinics in Dermatology 18(5):569–578

Aplasia cutis congenita

Synonym(s)Congenital ulcer of the newborn; congenitallocalized absence of skin; Streeter's spots;transient bullous dermolysis of newborn

DefinitionLocalized, congenital absence of a portionof skin

PathogenesisUnclear cause; possibly embryonic arrest inlocal skin development or intrauterine vas-cular abnormality or intrauterine trauma;hereditary factors possibly operative

Clinical manifestationStellate, linear, or oval, sharply demarcatedulceration, atrophic scar, or bulla, mostoften over the posterior scalp; multiplelesions occurring over the extremities,trunk, and buttocks; spontaneous healingin 1–3 months; with underlying bonydefects, healing in many months

Differential diagnosisIatrogenic injury from scalp electrode, etc.;congenital varicella; focal dermal hypopla-sia; epidermolysis bullosa; Volkmann’sischemic contracture

TherapySurgical reconstruction only for large, non-healing defects

ReferencesKruk-Jeromin J, Janik J, Rykala J (1998) Aplasia

cutis congenita of the scalp. Report of 16 cases. Dermatologic Surgery 24(5):549–553

Apocrine acne


Apocrine adenoma


Apocrine bromhidrosis

� Bromhidrosis

Apocrine cystadenoma

� Apocrine hidrocystoma

Apocrine hidrocystoma

Synonym(s)Apocrine cystadenoma; apocrine retentioncyst


Aquagenic pruritus 63

ADefinitionTumor consisting of a cystic proliferation ofapocrine secretory glands

PathogenesisMay be adenomatous cystic proliferation ofthe apocrine glands

Clinical manifestationAsymptomatic, solitary, translucent papuleor nodule, with a predilection for the eye-lid, particularly the inner canthus; cyst con-taining thin, clear, brownish fluid

Differential diagnosisEccrine hidrocystoma; basal cell carci-noma; epidermoid cyst; syringoma; milium

TherapyIncision and drainage, followed by surgicaldestruction of the cyst wall by light electro-desiccation and curettage or CO2 laservaporization; punch, shave, or ellipticalexcision

ReferencesSchleicher SM (1998) Multiple translucent facial

papules. Apocrine hidrocystoma. Archives of Dermatology 134(12):1627-1628, 1630–1631

Apocrine miliaria

� Fox-Fordyce disease

Apocrine poroma

� Poroma

Apocrine retention cyst


Apocrinitis


Aquagenic pruritus

Synonym(s)None

DefinitionRare genetic skin disorder causing pruritusupon contact with water or sudden temper-ature changes

PathogenesisElevated histamine levels during attacks;increased acetyl cholinesterase activity innerve fibers innervating sweat glands

Clinical manifestationIntense pruritus, with a pricking quality,which occurs immediately after bathing orswimming; provocation in some patientswith change in ambient temperature; symp-toms last for 1 hour and may flare withemotional or physical stress; no associatedskin signs

Differential diagnosisAquagenic urticaria; polycythemia vera-associated pruritus; xerosis-induced pruri-tus

TherapyPhotochemotherapy; UVB phototherapy;antihistamines, first generation; alkaliniza-tion of bath water; intramuscular triamci-nolone

Referencesdu Peloux Menage H, Greaves MW (1995) Aqua-

genic pruritus. Seminars in Dermatology 14(4):313–316


64 Aquagenic urticaria

Aquagenic urticaria

Synonym(s)None

DefinitionRare form of physical urticaria involvinghives caused by contact with water

PathogenesisSometimes occurring in patients with der-matographism; acetylcholine and hista-mine may be mediators

Clinical manifestationSmall urticarial wheals within minutes ofcontact with either fresh or sea water

Differential diagnosisAquagenic pruritus; dermatographism; coldurticaria; cholinergic urticaria

TherapyAntihistamines, first generation; photoche-motherapy; UVB phototherapy

ReferencesLuong KV, Nguyen LT (1998) Aquagenic urticaria:

report of a case and review of the literature. An-nals of Allergy, Asthma, & Immunology 80(6):483–485

Aqua glycolic lotion


Arachnidism

� Brown recluse spider bite

Arachnodactyly

DefinitionCondition involving abnormally long andslender hands and fingers, and often feetand toes may also have similar findings

ReferencesPyeritz RE (2000) The Marfan syndrome. Annual

Review of Medicine 51:481–510

Argyria

Synonym(s)Argyrosis

DefinitionDyspigmentation secondary to silver depo-sition in the skin

PathogenesisPigmentation secondary to silver deposi-tion in the dermis; metal-induced stimula-tion of melanogenesis in the epidermis;source of the silver via the oral route orthrough the skin after topical application

Clinical manifestationDiffuse, slate-gray pigmentation of the gin-giva and oral mucosa, sun-exposed skin,sclera, and nails

Argyria. Blue lunulae on the thumbs


Arteritis of the aged 65

ADifferential diagnosisCyanosis; diffuse melanosis from meta-static melanoma; hyperpigmentation fromother drugs, such as minocycline, gold, orphenothiazine derivative

TherapyDiscontinuation of exposure to silver;avoidance of sun exposure; chelating agentssuch as dimercaprol (BAL)

ReferencesHumphreys SD, Routledge PA (1998) The toxicol-

ogy of silver nitrate. Adverse Drug Reactions & Toxicological Reviews 17(2–3):115–143

Argyrosis

� Argyria

Arrid XX

� Aluminium chlorohydrate

Arsenical keratosis

Synonym(s)None

DefinitionPunctate keratoses of the palms and soles,occurring after long-term exposure to inor-ganic trivalent form of arsenic

PathogenesisInorganic arsenic retained in the body forlong periods after exposure, because ofpoor detoxification mechanisms; affectingmany enzymes by combining with sulfhy-dryl groups; acting as a cancer promoter,through its action on chromosomes

Clinical manifestationPunctate, non-tender, hard, yellowish, oftensymmetric, corn-like papules, mainly onthe palms and soles; pressure points com-monly involved; sometimes coalescing toform large, verrucous plaques

Differential diagnosisKeratosis palmaris et plantaris; clavus;wart; nevoid basal cell carcinoma syn-drome; porokeratosis; psoriasis of thepalms and soles; lichen planus; Darier dis-ease; Bazex syndrome; pityriasis rubra pila-ris

TherapyAcitretin; destructive modalities such aselectrosurgery, liquid nitrogen cryother-apy, and laser vaporization

ReferencesYerebakan O, Ermis O, Yilmaz E, Basaran E (2002)

Treatment of arsenical keratosis and Bowen's disease with acitretin. International Journal of Dermatology 41(2):84–87

Arteriovenous malformation

� Vascular malformation

Arteritis cranialis

� Temporal arteritis

Arteritis of the aged



66 Arteritis temporalis

Arteritis temporalis


Arthritis urethritica

� Reiter syndrome

Ascher syndrome

� Ascher’s syndrome

Ascher’s syndrome

Synonym(s)Ascher syndrome; double lip and nontoxicthyroid enlargement syndrome; struma-double lips syndrome; thyroid blepharoch-alasis syndrome; Fuchs’ syndrome III;Laffer-Ascher syndrome

DefinitionDisorder consisting of blepharochalasis,double lip, and non-toxic goiter

PathogenesisUnknown

Clinical manifestationBlepharochalasis (excessive upper lid skin);duplication of the upper lip; euthyroidgoiter

Differential diagnosisGrave’s disease; angioedema

TherapySurgical correction of excess eyelid skin andlip

ReferencesSanchez MR, Lee M, Moy JA, Ostreicher R (1993)

Ascher syndrome: a mimicker of acquired an-gioedema. Journal of the American Academy of Dermatology 29(4):650–651

Ash-leaf macule

DefinitionSharply circumscribed, round-to-oval areaof macular hypopigmentation seen at birthin patients with tuberous sclerosis

ReferencesArbuckle HA, Morelli JG (2000) Pigmentary dis-

orders: update on neurofibromatosis-1 and tu-berous sclerosis. Current Opinion in Pediatrics 12(4):354–358

Ashy dermatosis

Synonym(s)Ashy dermatosis of Ramirez; erythema dys-chromicum perstans; dermatosiscenicienta; erythema chronicum figuratummelanodermicum; lichen pigmentosus

DefinitionEruption of gray-blue macules over thetrunk; closely linked to lichen planus

PathogenesisUnknown

Clinical manifestationAsymptomatic, gray-blue patches of varia-ble shape and size, distributed symmetri-cally on the face, trunk, and upper extremi-ties; elevated, erythematous border in theearly stages; oral cavity and genitals spared

Differential diagnosisLichen planus; lichenoid drug eruption;tuberculoid leprosy; pinta; hemochromato-sis


Asteatotic eczema 67

ATherapyClofazimine 100 mg PO every other day ifunder 40 kg in weight; clofazimine 100 mgevery day if greater than 40 kg in weight;ultraviolet exposure; ultraviolet avoidance;antibiotics; antihistamines; psychotherapy

ReferencesOsswald SS, Proffer LH, Sartori CR (2001) Ery-

thema dyschromicum perstans: a case report and review. Cutis 68(1):25–28

Ashy dermatosis of Ramirez

� Ashy dermatosis

Asteatosis

Synonym(s)Dry skin; xerosis; winter itch

DefinitionIrritation caused by lack of moisture in theskin

PathogenesisPhysiologic process with aging; seen moreoften in the winter, with cold air outsideand heated air inside causing a decrease inhumidity

Clinical manifestationGeneralized pruritus, often worse afterbathing; most common on the lower legs,arms, flanks, and thighs; may be associatedwith mild erythema and scaliness

Differential diagnosisOther causes of generalized pruritus: sca-bies; atopic dermatitis; drug reaction;obstructive hepatobiliary disease; end-stagerenal disease; polycythemia vera; Hodg-kin’s disease; thyroid disease; hyperparathy-roidism; psychogenic pruritus

TherapyDecreased bathing; use of soap substitutessuch as bath gels; application of emollientsat least twice daily during the wintermonths; antihistamines, first generation, fornighttime sedation

� Xerosis

ReferencesBeacham BE (1993) Common dermatoses in the

elderly. American Family Physician 47(6):1445–1450

Asteatotic dermatitis

� Asteatotic eczema

Asteatotic eczema

Synonym(s)Asteatotic dermatitis; eczema craquelé;eczema craquelatum; xerotic eczema;eczema hiemalis; eczema fendille; etatcraquelé

DefinitionPruritic, cracked, and fissured skin occur-ring most commonly on the shins of eld-erly patients, caused by lack of moisture inthe skin

PathogenesisPhysiologic process with aging; seen moreoften in the winter, with cold air outsideand heated air inside causing a decrease inhumidity; loss of water by stratum cor-neum causing cells to shrink and creatingfine fissures; eczematous changes resultingfrom patients rubbing and scratching thesepruritic areas

Clinical manifestationMinimally scaly, red, cracked, and or fis-sured skin, giving the appearance of a“cracked pot”; most commonly involving


68 Ataxia-telangiectasia

the pretibial areas, but also the thighs,hands and trunk; generalized pruritus,often worse after bathing

Differential diagnosisIchthyosis; atopic dermatitis; nummulareczema; stasis dermatitis; contact dermati-tis; mycosis fungoides; other causes of gen-eralized pruritus: scabies; atopic dermati-tis; drug reaction; obstructive hepatobil-iary disease; end-stage renal disease;polycythemia vera; Hodgkin’s disease; thy-roid disease; hyperparathyroidism; psycho-genic pruritus

TherapyDecreased bathing; use of soap substitutessuch as bath gels; application of emollientsat least twice daily during the wintermonths; mid potency topical corticosteroidointment; antihistamines, first generation,for nighttime sedation



Ataxia-telangiectasia

Synonym(s)Louis-Bar syndrome; Boder-Sedgwick syn-drome

DefinitionAutosomal, recessive, multisystem disordercharacterized by progressive neurologicalimpairment, cerebellar ataxia, variableimmunodeficiency, impaired organ matura-tion, x-ray hypersensitivity, ocular andcutaneous telangiectasia, and a predisposi-tion to malignancy

PathogenesisUnclear; possibly associated with dysregu-lation of the immunoglobulin gene super-family, which includes genes for T-cell

receptors; abnormal sensitivity to x-raysand certain radiomimetic chemicals, possi-bly leading to chromosomal abnormalities,infections, and malignancies

Clinical manifestationOcular and cutaneous telangiectasia; neu-rological abnormalities, mainly ataxia,abnormal eye movements, and chore-oathetosis

Differential diagnosisTelangiectatic diseases: hereditary hemor-rhagic telangiectasia; chronic liver disease;benign essential telangiectasia; sun dam-age; neurologic disorders; Friedreich dis-ease; cerebral palsy; familial spinocerebel-lar atrophies; GM1 and GM2 gangliosi-doses; progressive rubella panencephalitis;subacute sclerosing panencephalitis;postinfectious encephalomyelitis; cerebel-lar tumor

TherapyNo effective therapy

ReferencesGatti RA (1995) Ataxia-telangiectasia. Dermato-

logic Clinics 13(1):1–6

Atheroma

� Epidermoid cyst

Athlete’s feet

� Tinea pedis

Atopic dermatitis

Synonym(s)Atopic eczema; infantile eczema; Besnier'sprurigo


Atrophic parapsoriasis 69

A

DefinitionDisease starting in early infancy and char-acterized by pruritus, eczematous lesions,dry skin, and an association with otheratopic diseases (asthma, allergic rhinitis,urticaria)

PathogenesisAbnormality of T helper type 2 (TH2) cells,resulting in increased production of inter-leukin 4 (IL-4) and increased IgE; stratumcorneum lipid defect, leading to increasedtransepidermal water loss

Clinical manifestationMarked pruritus, often starting in the firstfew months of life; asthma or hay fever or ahistory of atopic disease in a first-degreerelative; dry skin; lichenified plaques withepithelial disruption, occurring on the facein infancy, in the flexural creases, trunk,and diaper area by 1 year of age, and overthe distal extremities later in life; scalpinvolvement, usually after age 3 months

Differential diagnosisSeborrheic dermatitis; contact dermatitis;stasis dermatitis; nummular eczema; sca-bies; mycosis fungoides; dermatophytosis

TherapyMid potency topical corticosteroids�; pred-nisone for temporary therapy of severeflares; pimecrolimus 1% cream; tacrolimus0.3% or 1% ointment; azathioprine; cyclo-sporine; antihistamines, first generation, fornighttime sedation UVB phototherapy;

photochemotherapy (PUVA); evening prim-rose oil; Chinese herbs; emollients appliedat least twice daily, particularly during thewinter months

ReferencesTofte SJ, Hanifin JM (2001) Current management

and therapy of atopic dermatitis. Journal of the American Academy of Dermatology 44(1 Sup-pl):S13–16

Atopic eczema

� Atopic dermatitis

Atopy

Synonym(s)None

DefinitionPredisposition to develop allergic reac-tions, often genetically determined andinvolving the production of IgE antibodies

ReferencesMacLean JA, Eidelman FJ (2001) The genetics of

atopy and atopic eczema. Archives of Derma-tology 137(11):1474–1476

Atrofodermia idiopatica progressiva

� Atrophoderma of Pasini and Pierini

Atrophic parapsoriasis

� Large plaque parapsoriasis

Atopic dermatitis. Lichenified, red plaque with erosions in the antecubital fossa


70 Atrophie brilliante

Atrophie brilliante

� Confluent and reticulated papillo-matosis


Synonym(s)Idiopathic atrophoderma of Pasini andPierini; atrophodermia idiopatica progres-siva

DefinitionAsymptomatic atrophy of the skin charac-terized by single or multiple, defined,depressed areas of skin

PathogenesisPossibly an end result of morphea; possiblyrelated to spirochete infection (in Europe)

Clinical manifestationPresenting as asymptomatic, slightly ery-thematous plaque or plaques on the trunk;lesions developing slate-gray to brown pig-mentation, sharp peripheral border, andcentral depression

Differential diagnosisMorphea; lichen sclerosus; skin atrophyfrom steroid injection; anetoderma; post-inflammatory hyperpigmentation

TherapyDoxycycline; amoxicillin; hyperpigmenta-tion component: Q-switched alexandritelaser

ReferencesBuechner SA, Rufli T (1994) Atrophoderma of

Pasini and Pierini. Clinical and histopathologic findings and antibodies to Borrelia burgdorferi in thirty-four patients. Journal of the American Academy of Dermatology 30(3):441–446

Atrophoderma pigmentosum


Atrophoderma reticulatum

� Keratosis pilaris atrophicans

Atrophoderma vermiculatum

� Keratosis pilaris atrophicans� Ulerythema ophryogenes

Atypical fibroxanthoma

Synonym(s)Paradoxical fibrosarcoma; pseudosarcoma;pseudosarcomatous reticulohistiocytoma;pseudosarcomatous dermatofibroma

DefinitionRapidly enlarging tumor, arising in chroni-cally sun-exposed skin, with histologic fea-tures suggesting a malignant connective tis-sue neoplasm, but usually benign clinicalcourse

PathogenesisSolar radiation and prior X-irradiation pos-sible predisposing factors

Clinical manifestationFirm, solitary, eroded or ulcerated papuleor nodule on sun-exposed skin, particu-larly the ear, nose, and cheek; most com-mon in elderly patients


Atypical mole syndrome 71

ADifferential diagnosisSquamous cell carcinoma; pyogenic granu-loma; melanoma; basal cell carcinoma;Merkel cell carcinoma; cutaneous metasta-sis; leiomyosarcoma; dermatofibrosarcomaprotuberans

TherapyMohs micrographic surgery�; ellipticalexcision; destruction by electrodesiccationand curettage

ReferencesDavis JL, Randle HW, Zalla MJ, Roenigk RK,

Brodland DG (1997) A comparison of Mohs mi-crographic surgery and wide excision for the treatment of atypical fibroxanthoma. Dermato-logic Surgery 23(2):105–110

Atypical lipoma

� Liposarcoma

Atypical lipomatous tumors

� Liposarcoma

Atypical melanocytic nevus

� Atypical mole

Atypical mole

Synonym(s)Active junctional nevus; atypical melano-cytic nevus; B-K mole, Clark's nevus;atypical mole syndrome; dysplastic mole;dysplastic nevus

DefinitionBenign melanocytic growth, possibly shar-ing some of the clinical or microscopic fea-tures of melanoma, but not a melanoma

PathogenesisGenetic component in some patients(melanoma-prone families; familial atypi-cal mole syndrome); sunlight exposure pos-sibly a factor

Clinical manifestationVariable features, with some or all of thefollowing: asymmetrical conformation;irregular border which can fade impercepti-bly into the surrounding skin; variable col-oration, with shades of tan, brown, black;and red; diameter > 6 mm; elevated centerand feathered, flat border, giving the lesionthe appearance of a fried egg

Differential diagnosisMelanoma; compound nevus; seborrheickeratosis; dermatofibroma; wart

TherapyAvoidance of excessive sun exposure; use ofsunscreen with a sun protective factor of 15or greater; evaluation of other family mem-bers for evidence of atypical moles; base-line photographs of entire skin surface, ifpossible

ReferencesSlade J, Marghoob AA, Salopek TG, Rigel DS, Kopf

AW, Bart RS (1995) Atypical mole syndrome: risk factor for cutaneous malignant melanoma and implications for management. Journal of the American Academy of Dermatology 32(3):479–494

Atypical mole syndrome

� Atypical mole


72 Audry’s glands

Audry’s glands

� Fordyce’s disease

Auranofin

Trade name(s)Ridaura

Generic availableNo

Drug classAnti-rheumatic

Mechanism of actionInhibition of complement and lysosomalenzymes; normalization of defective Lang-erhans cell antigen presentation



Common side effectsCutaneous: skin eruption, stomatitis, pruri-tus, glossitisGastrointestinal: diarrhea, abdominal painLaboratory: anemia, leukopenia, proteinu-riaNeurologic: change in taste sensationOcular: keratitis

Serious side effectsBone marrow: agranulocytosisNeurologic: seizuresPulmonary: pneumonitisRenal: renal failure, nephrotic syndrome

Drug interactionsAtovaquone/proguanil

Auranofin. Dermatologic indications and dosage


Cicatricial pemphigoid

3 mg PO twice daily Initial: 0.1 mg per kg daily in 1–2 divided doses; usual maintenance: 0.15 mg/kg/day in 1–2 divided doses; maximum: 0.2 mg/kg/day in 1–2 divided doses

Epidermolysis bullosa acquisita

3 mg PO twice daily Initial: 0.1 mg per kg daily; usual maintenance: 0.15 mg/kg/day in 1–2 divided doses; maximum: 0.2 mg/kg/day in 1–2 divided doses

Lupus erythematosus 3 mg PO twice daily Initial: 0.1 mg per kg daily in 1–2 divided doses; usual maintenance: 0.15 mg/kg/day in 1–2 divided doses; maximum: 0.2 mg/kg/day in 1–2 divided doses

Pemphigus vulgaris 3 mg PO twice daily Initial: 0.1 mg per kg daily in 1–2 divided doses; usual maintenance: 0.15 mg/kg/day in 1–2 divided doses; maximum: 0.2 mg/kg/day in 1–2 divided doses


Aurothioglucose 73

AContraindications/precautionsHypersensitivity to drug class or compo-nent; pulmonary fibrosis; bone marrowaplasia; caution with impaired liver or renalfunction

ReferencesPapp KA, Shear NH (1991) Systemic gold therapy.

Clinics in Dermatology 9(4):535–551


Synonym(s)Frey’s syndrome; Baillarger's syndrome;Dupuy's syndrome; salivosudoriparoussyndrome; sweating gustatory syndrome;gustatory sweating

DefinitionGustatory sweating secondary to auriculo-temporal nerve injury

PathogenesisMisdirection of parasympathetic fibers,which migrate into the postganglionic sym-pathetic fibers to innervate the sweat glands

Clinical manifestationFlushing or sweating on one side of the facewhen certain foods are eaten

Differential diagnosisGustatory sweating from diabetic neuropa-thy or post-herpetic neuralgia; Horner’ssyndrome; lacrimal sweating; harlequinsyndrome

TherapySurgical: tympanic neurectomy for severesymptoms; perineural alcohol injectionMedical: scopolamine 3–5% cream appliedtwice daily; aluminium chloride

� Gustatory sweating

ReferencesKaddu S, Smolle J, Komericki P, Kerl H (2000) Au-

riculotemporal (Frey) syndrome in late child-

hood: an unusual variant presenting as gustatory flushing mimicking food allergy. Pediatric Dermatology 17(2):126–128

Aurothioglucose

Trade name(s)Solganol

Generic availableNo

Drug classAnti-rheumatic

Mechanism of actionInhibition of complement and lysosomalenzymes; normalization of defective Lang-erhans cell antigen presentation

Dosage formIntramuscular injection


Common side effectsCutaneous: stomatitis, glossitis, skin erup-tion, pruritusGastrointestinal: diarrhea, abdominal pain,dyspepsia, change in taste sensationLaboratory: proteinuria, anemia, leukope-niaNeurologic: change in taste sensationOcular: keratitis

Serious side effectsLaboratory: agranulocytosisNeurologic: seizuresPulmonary: pneumonitisRenal: renal failure, nephrotic syndrome

Drug interactionsAtovaquone/proguanil


74 Auspitz sign

Contraindications/precautionsHypersensitivity to drug class or compo-nent; pulmonary fibrosis; bone marrowaplasia; caution with impaired liver or renalfunction

ReferencesPapp KA, Shear NH (1991) Systemic gold therapy.


Auspitz sign

DefinitionBleeding points appearing when overlyingscale removed physically from a lesion ofpsoriasis

ReferencesBernhard JD (1997) Clinical pearl: auspitz sign in

psoriasis scale. Journal of the American Acade-my of Dermatology 36(4):621

Autoeczematization

� Id reaction

Autoerythrocyte sensitization

� Autoerythrocyte sensitization syn-drome

Aurothioglucose. Dermatologic indications and dosage



25–50 mg IM once weekly Initial – 0.25 mg per kg per dose first week; increment at 0.25 mg per kg per dose increasing with each weekly dose; maintenance – 0.75–1 mg per kg per dose weekly, not to exceed 25 mg per dose



Lupus erythematosus; pemphigus vulgaris; cicatricial pemphigoid; epidermolysis bullosa acquisita


Pemphigus vulgaris 25–50 mg IM once weekly Initial – 0.25 mg per kg per dose first week; increment at 0.25 mg per kg per dose increasing with each weekly dose; maintenance – 0.75–1 mg per kg per dose weekly, not to exceed 25 mg per dose


Azathioprine 75

AAutoerythrocyte sensitization syndrome

Synonym(s)Gardner-Diamond syndrome; autoeryth-rocyte sensitization; psychogenic purpura;purpura autoerythrocytica

DefinitionPurpuric disorder in women, characterizedby painful ecchymotic patches, unrelated tovascular or clotting abnormalities

PathogenesisPossibly an immune-mediated reaction;psychological issues in the patients possi-bly the main causative factor

Clinical manifestationPainful ecchymoses, often appearing afterminor trauma, usually over the extremitiesand trunk; lesions appearing in crops, andlasting for weeks to months

Differential diagnosisAnaphylactoid purpura; Ehlers-Danlos syn-drome; child abuse; factitial purpura; amy-loidosis; thrombotic thrombocytopenicpurpura; solar purpura; leukemia

TherapyMedroxyprogesterone acetate 10 mg PO perday or 150 mg intramuscularly once permonth; prednisone; antihistamines, firstgeneration

ReferencesBerman DA, Roenigk HH, Green D (1992) Auto-

erythrocyte sensitization syndrome (psycho-genic purpura). Journal of the American Academy of Dermatology 27(5 Pt 2):829–832

Autoimmune alopecia

� Alopecia areata

Autoimmune dermatosis of pregnancy

� Herpes gestationis

Autosensitization

� Id reaction

Autosomal dominant ichthyosis

� Ichthyosis vulgaris

Autumnal fever

� Leptospirosis

Axillary freckling

DefinitionBrown macules in the axillary vault, presentin more than 90 percent of people with neu-rofibromatosis, type 1

ReferencesWainer S (2002) A child with axillary freckling

and cafe au lait spots. Canadian Medical Asso-ciation Journal 167(3):282–283

Azathioprine

Trade name(s)Imuran


76 Azathioprine


Drug classAntimetabolite; immunosuppressant

Mechanism of actionActive metabolite is purine analog, whichinhibits DNA and RNA synthesis and hasimmunosuppressive activity

Azathioprine. Dermatologic indications and dosage


Atopic dermatitis 2–3 mg per kg PO daily 2–3 mg per kg PO daily

Behçet’s disease Corticosteroid sparing function; 2–3 mg per kg PO daily

Corticosteroid sparing function; 2–3 mg per kg PO daily

Bullous pemphigoid Corticosteroid sparing function; 2–3 mg per kg PO daily


Chronic actinic dermatitis






Fogo selvagem 2–3 mg per kg PO daily 2–3 mg per kg PO daily

Leukocytoclastic vasculitis



Lupus erythematosus, acute



Mixed connective tissue disease

2–3 mg per kg PO daily 2–3 mg per kg PO daily

Paraneoplastic pemphigus



Pemphigus foliaceus 2–3 mg per kg PO daily 2–3 mg per kg PO daily

Pemphigus vulgaris Corticosteroid sparing function; 2–3 mg per kg PO daily


Persistent light reaction



Polyarteritis nodosa Corticosteroid sparing function; 2–3 mg per kg PO daily


Pyoderma gangrenosum



Relapsing polychondritis


Sarcoidosis 2–3 mg per kg PO daily 2–3 mg per kg PO daily

Sulzberger-Garbe syndrome


Weber-Christian disease


Wegener’s granulomatosis




Azelaic acid 77

ADosage form50 mg tablet


Common side effectsCutaneous: alopecia, skin eruptionGastrointestinal: nausea and vomiting,diarrhea, dyspepsiaLaboratory: elevated liver enzymes

Serious side effectsCutaneous: hypersensitivity reactionGastrointestinal: hepatotoxicity, pancreati-tisImmune: immunosuppressionNeoplastic: increased risk of neoplasm,particularly lymphoma

Drug interactionsACE inhibitors; allopurinol; cisplatin; cyto-toxic chemotherapeutic agents; interferonalfa 2a; interferon beta; mycophenolatemofetil; warfarin; zidovudine

Contraindications/precautionsHypersensitivity to drug class or compo-nent; pregnancy; caution if patient has lowlevels or lacks thiopurine methyltrans-ferase (measure enzyme level before start-ing therapy); caution if impaired liver func-tion

ReferencesSilvis NG (2001) Antimetabolites and cytotoxic

drugs. Dermatologic Clinics 19(1):105–118

Azelaic acid

Trade name(s)Azelex; Finacea

Generic availableNo

Drug classAnti-acne; anti-rosacea

Mechanism of actionMay be related to antimicrobial effects

Dosage form15% cream, 20% cream


Common side effectsCutaneous: pruritus, burning sensation,dryness, skin eruption




Azelaic acid. Dermatologic indications and dosage



Melasma Apply twice daily Apply twice daily

Postinflammatory hyperpigmentation




78 Azithromycin

ReferencesNguyen QH, Bui TP (1995) Azelaic acid: pharma-

cokinetic and pharmacodynamic properties and its therapeutic role in hyperpigmentary disorders and acne. International Journal of Dermatology 34(2):75–84

Azithromycin

Trade name(s)Zithromax

Generic availableNo

Drug classMacrolide antibiotic

Mechanism of actionInhibits protein synthesis of sensitive bacte-rial organisms

Dosage form250 mg, 500 mg tablet; powder for oral sus-pension


Common side effectsCutaneous: skin eruption, vaginitisGastrointestinal: nausea, vomiting, abdom-inal pain, diarrhea, anorexia

Serious side effectsCutaneous: anaphylaxis, Stevens-Johnsonsyndrome, toxic epidermal necrolysis Gastrointestinal: pseudomembranous coli-tis, cholestatic jaundice

Drug interactionsAntacids; oral contraceptives; warfarin; dig-oxin

Azithromycin. Dermatologic indications and dosage


Bacillary angiomatosis 500 mg PO on day 1; 250 mg PO on days 2–5

Not indicated in those < 45 kg in weight; 500 mg PO on day 1; 250 mg PO on days 2–5

Bartonellosis 500 mg PO on day 1; 250 mg PO on days 2–5


Cellulitis 500 mg PO on day 1; 250 mg PO on days 2–5


Chancroid 1 gm PO for 1 dose Not indicated in those < 45 kg in weight; 20 mg per kg PO for 1 dose

Ecthyma 500 mg PO on day 1; 250 mg PO on days 2–5


Furuncle 500 mg PO on day 1; 250 mg PO on days 2–5


Impetigo 500 mg PO on day 1; 250 mg PO on days 2–5


Trench fever 250-500 mg PO for 4 weeks Not indicated in those < 45 kg; 250 mg PO daily for 4 weeks


Azul 79

A

Contraindications/precautionsHypersensitivity to drug class or compo-nent; caution in those with impaired liverfunction; do not use concomitantly withterfenadine or astemizole

ReferencesAlvarez-Elcoro S, Enzler MJ (1999) The mac-

rolides: erythromycin, clarithromycin, and azi-thromycin. Mayo Clinic Proceedings 74(6):613–634

Azole antifungal agents

Trade name(s)Generic in parentheses:Exelderm (sulconazole); Lamisil AT (terbin-afine); Lotrimin; Mycelex (clotrimazole);Micatin (miconazole); Nizoral (ketocona-zole); Oxistat (oxiconazole); Spectazole(econazole)


Drug classAzole antifungal agents

Mechanism of actionCell wall ergosterol inhibition secondary toblockade of 14α-demethlyation of lanos-terol

Dosage formCream; solution; lotion


Common side effectsCutaneous: skin eruption, pruritus




ReferencesWeinstein A, Berman B (2002) Topical treatment

of common superficial tinea infections. Ameri-can Family Physician 65(10):2095–2102

Azul

� Pinta

Azole antifungal agents. Dermatologic indications and dosage


Angular cheilitis Apply twice daily for 2–4 weeks Apply twice daily for 2–4 weeks

Cutaneous candidiasis Apply twice daily for 2–4 weeks Apply twice daily for 2–4 weeks

Majocchi granuloma Apply twice daily for 4–8 weeks Apply twice daily for 4–8 weeks

Onychomycosis Apply twice daily for 2–4 weeks Apply twice daily for 2–4 weeks

Tinea corporis Apply twice daily for 2–4 weeks Apply twice daily for 2–4 weeks

Tinea cruris Apply twice daily for 2–4 weeks Apply twice daily for 2–4 weeks

Tinea faciei Apply twice daily for 2–4 weeks Apply twice daily for 2–4 weeks

Tinea nigra Apply twice daily for 2–4 weeks Apply twice daily for 2–4 weeks

Tinea pedis Apply twice daily for 2–4 weeks Apply twice daily for 2–4 weeks

Tinea versicolor Apply twice daily for 2–4 weeks Apply twice daily for 2–4 weeks

White piedra Apply twice daily for 2–4 weeks Apply twice daily for 2–4 weeks


B

B-K mole

�

Atypical mole

Bacillary ailuronosis

�

Bacillary angiomatosis

Bacillary angiomatosis

Synonym(s)

Epithelioid angiomatosis

;

bartonellosis

;

bacillary ailuronosis

;

disseminated cat-scratch disease

Definition

Infection caused by closely related gram-negative bacteria, Bartonella henselae andBartonella quintana, occurring mostly inimmunocompromised patients

Pathogenesis

Gram-negative bacillary infection resultsfrom exposure to flea-infested cats with

B

henselae and the human body louse for

B

quintana


Globular angiomatous papules or nodulesresembling pyogenic granulomas; viola-

ceous nodules resembling Kaposi’s sar-coma; lichenoid violaceous plaques; subcu-taneous papules or nodules, with or with-out ulceration


Kaposi’s sarcoma; glomangioma; verrugaperuana; angiokeratoma; hemangioma;pyogenic granuloma; gram-positive bacte-rial abscess; nodal myofibromatosis;melanoma

Therapy

Erythromycin

�

; azithromycin; clarithromy-cin; doxycycline

References

Manders SM (1996) Bacillary angiomatosis. Clin-ics in Dermatology 14(3):295–299

Bacillary peliosis

�

Bartonellosis

Bacitracin

Trade name(s)

Bacitracin as single agent: Baciguent; baci-tracin as one component of a multi-agentpreparation: Betadine antibiotic ointment;Gold Bond Triple Action; Mycitracin;Neosporin; Polysporin; Spectrocin Plus


82 Bagdad boil

Generic available

Yes

Drug class

Antibiotic

Mechanism of action

Inhibits bacterial cell wall synthesis

Dosage form

Cream; ointment


See table

Common side effects

Cutaneous:

contact dermatitis


None

Drug interactions

None


Hypersensitivity to drug class or compo-nent

References

Bass, JW, Chan DS, Creamer KM, Thompson MW, Malone FJ, Becker TM, Marks SN (1997) Com-parison of oral cephalexin, topical mupirocin and topical bacitracin for treatment of impeti-go. Pediatric Infectious Disease Journal 16(7):708–710

Bagdad boil

�

Leishmaniasis, cutaneous

Baillarger's syndrome

�


Balanitis

Synonym(s)

Balanoposthitis

Definition

Inflammation of the foreskin and head ofthe penis

References

Bunker CB (2001) Topics in penile dermatology. Clinical & Experimental Dermatology 26(6):469–479

Balanitis circumscripta plasmacellularis

�

Zoon balanitis


Synonym(s)

Lichen sclerosus of the penis

;

male genitallichen sclerosus

;

lichen sclerosus et atrophi-cus of the penis

;

penile lichen sclerosus

Bacitracin. Dermatologic indications and dosage


Impetigo Apply twice per day for 7 days Apply twice per day for 7 days

Postoperative wound infection prophylaxis

Apply twice per day for 7 days Apply twice per day for 7 days


Bannayan-Riley-Ruvalcaba syndrome 83

B

Definition

Chronic, progressive, sclerosing, inflamma-tory dermatosis of the penis and prepuce

Pathogenesis

Unknown; minor relationship with autoim-mune disorders


Presents with soreness, burning sensation,mild erythema and hypopigmentation; asdisease progresses, single or multiple dis-crete erythematous papules or macules coa-lescing into atrophic ivory, white, or purple-white patches or plaques, which may erode;possible development of vesiculation; possi-ble phimosis occurring in uncircumcisedmen; occasional signs of lichen sclerosus atother skin sites


Plasma cell balanitis; candidiasis; lichenplanus; psoriasis; vitiligo; Reiter syndrome;erythroplasia of Queyrat

Therapy

Surgical therapy: circumcision; laser vapor-izationMedical therapy: superpotent topical corti-costeroids; testosterone propionate 1% oint-ment applied twice daily; acitretin

�

Lichen sclerosus

References

Das S, Tunuguntla HS (2000) Balanitis xerotica obliterans – A review. World Journal of Urology 18(6):382–387

Balanoposthitis

�

Balanitis

Baldness

�

Alopecia

Bamboo hair

�

Trichorrhexis invaginata

Bancroftian filariasis

�

Filariasis

Bannayan syndrome

�

Bannayan-Riley-Ruvalcaba syn-drome

Bannayan-Riley-Ruvalcaba syndrome

Synonym(s)

Bannayan-Zonana syndrome

;

Riley-Smithsyndrome

;

Ruvalcaba-Myhre syndrome

;

Ruvalcaba-Myhre-Smith syndrome

;

Banna-yan syndrome

;

Cowden/Bannayan-Riley-Ruvalcaba overlap syndrome

;

PTEN hama-rtoma tumor syndrome

;

macrocephaly

;

pseudopapilledema

;

multiple hemangiom-ata syndrome

;

multiple lipomas

Definition

Disease characterized by hamartomatouspolyps of the small and large intestine,macrocephaly, lipomas, hemangiomas, thy-roid abnormalities, and freckling of thepenis

Pathogenesis

Autosomal dominant inheritance; muta-tion in the tumor suppressor gene, PTEN


84 Bannayan-Zonana syndrome


Hamartomatous polyps of the small andlarge intestine; macrocephaly; lipomas;hemangiomas; thyroid abnormalities;penile freckling; developmental delay;increased risk for both benign and malig-nant tumors


Cowden’s syndrome; Gardner’s syndrome;multiple lentigines syndrome

Therapy

Increased breast, thyroid, and colon cancersurveillance; surgical excision of lipomasand hemangiomas for cosmetic purposesonly

References

Fargnoli MC, Orlow SJ, Semel-Concepcion J, Bo-lognia JL (1996) Clinicopathologic findings in the Bannayan-Riley-Ruvalcaba syndrome. Ar-chives of Dermatology 132(10):1214–1218

Bannayan-Zonana syndrome

�

Bannayan-Riley-Ruvalcaba syn-drome

Barber's itch

�

Sycosis barbae

Barber-Say syndrome

Synonym(s)

Say syndrome

Definition

Disease entity consisting of hypertrichosis,xerosis, cutis laxa, dysmorphic facial fea-tures, and eye changes

Pathogenesis

Autosomal recessive inheritance


Hypertrichosis over the upper trunk andface; xerosis; generalized cutis laxa; macros-tomia; opacification of the corneas; varia-ble nystagmus


Cone-rod congenital amaurosis; ablepha-ron-macrostomia syndrome; Turner’s syn-drome; Brachmann-de Lange syndrome;Sanfilippo syndrome; Hunter’s syndrome;leprechaunism

Therapy

No effective therapy

References

Martinez Santana S, Perez Alvarez F, Frias JL, Martinez-Frias ML (1993) Hypertrichosis, atrophic skin, ectropion, and macrostomia (Barber-Say syndrome): report of a new case. American Journal of Medical Genetics 47(1):20–23

Barlow’s disease

Synonym(s)

Möller-Barlow disease

;

Barlow’s syndrome

;

Cheadle-Möller-Barlow syndrome

;

Moeller's disease

;

infantile scurvy

;

vitaminC deficiency syndrome

Definition

Vitamin C deficiency disease in children,manifested by gingival lesions, hemor-rhage, arthralgia, loss of appetite, and list-lessness

Pathogenesis

Vitamin C deficiency, after at least 3 monthsof severe or total lack of vitamin C, result-ing in defective collagen synthesis anddefective folic acid and iron utilization


Bartonellosis 85

B


Perifollicular hyperkeratotic papules, sur-rounded by a hemorrhagic halo; hairstwisted like corkscrews and possibly frag-mented; submucosal gingival bleeding, sub-periosteal hemorrhage, arthralgia; ano-rexia; listlessness; exophthalmos and con-junctival hemorrhage; poor wound healing


Vasculitis; child abuse; coagulation abnor-malities with leukemia; platelet abnormali-ties, etc.; deep vein thrombosis; throm-bophlebitis

Therapy

Ascorbic acid 150–300 mg per day for 1month

References

Ghorbani AJ, Eichler C (1994) Scurvy. Journal of the American Academy of Dermatology 30(5 Pt 2):881–883

Barlow’s syndrome

�

Barlow’s disease

Barraquer-Simons disease

�

Progressive lipodystrophy

Barraquer-Simons syndrome

�


Bartonellosis

Synonym(s)

Cat scratch disease

;

catscratch disease

;

trench fever

;

urban trench fever

;

bacillarypeliosis

;

Parinaud oculoglandular syn-drome

;

Parinaud's oculoglandular syn-drome

;

Oroya fever

;

Carrión disease

;

Carrión's disease

;

verruga peruana; benignlymphoreticulosis

DefinitionInfections caused by species belonging tothe bacterial genus Bartonella

PathogenesisBartonella henselae found in associationwith both domestic and feral cats and pre-sumably passed from cat to human; Bar-tonella quintana spread via human bodylouse

Clinical manifestationCat scratch disease: papule or pustuledeveloping 5–10 days after exposure; fever;malaise; lymphadenopathyOroya fever (verruga peruana): onset offever 3–12 weeks after a sand fly bite; cropsof small papules enlarging and healing byfibrosis over several months

Differential diagnosisLymphoma; leukemia; deep fungal infec-tion; tuberculosis; plague; lymphogranu-loma venereum; AIDS; syphilis; denguefever; malaria; babesiosis

TherapyDoxycycline; erythromycin; azithromycin;clarithromycin

� Bacillary angiomatosis

ReferencesMaguina C, Gotuzzo E (2000) Bartonellosis: new

and old. Infectious Disease Clinics of North America 14(1):1–22


86 Bart’s syndrome

Bart’s syndrome

Synonym(s)None

DefinitionSubtype of dominant dystrophic epidermol-ysis bullosa with congenital localizedabsence of skin, nail abnormalities, andblistering

PathogenesisMutation of the COLA7A1 gene, resulting inthe production of poorly formed anchoringfibrils at the skin’s basement membranezone

Clinical manifestationCongenital erosions of the lower extremi-ties, which heal with hairless scars; trauma-induced blistering; absent or dystrophicnails; mucous membrane erosions only inearly life

Differential diagnosisAplasia cutis congenita; epidermolysis bul-losa simplex; junctional epidermolysis bul-losa; child abuse

TherapyHydrocolloid dressings to erosions; petrola-tum between toes to minimize scarring

ReferencesAmichai B, Metzker A (1994) Bart's syndrome. In-

ternational Journal of Dermatology 33(3):161–163

Basal cell carcinoma

Synonym(s)Basal cell epithelioma; basalioma; Jacob’sulcer; rodent ulcer

DefinitionCutaneous neoplasm arising from pluripo-tential cells of the epidermis or its append-ages

PathogenesisEarly, intense sun exposure possibly caus-ing p53 tumor suppressor gene mutations,allowing unrestricted proliferation

Clinical manifestationNodular variant: pearly, translucent papulewith central depression, erosion, or ulcera-tion; rolled borders; telangiectasia on thesurfacePigmented variant: flecks of gray or bluepigment in addition to features describedfor nodular variantSuperficial variant: pink-to-brown, scalyplaque or papule, often with annular con-figurationMorpheaform variant: poorly demarcated,sclerotic plaque or papule

Differential diagnosisSquamous cell carcinoma; nevus; fibrouspapule; wart; appendage tumor; seborrheickeratosis; sebaceous gland hyperplasia;Bowen’s disease

TherapyPrimary tumor in anatomically insensitivesites: destruction by electrodesiccation andcurettage; elliptical excision; cryotherapy;orthovoltage radiation therapy; fluorour-acil cream

Basal cell carcinoma. Papule with rolled margins and central erosion on the nasal bridge


Basan syndrome 87

B

Recurrent tumor or tumors in anatomicallysensitive sites: Mohs micrographic sur-gery�

ReferencesThissen MR, Neumann MH, Schouten LJ (1999) A

systematic review of treatment modalities for primary basal cell carcinomas. Archives of Der-matology 135(10):1177–1183

Basal cell epithelioma

� Basal cell carcinoma

Basal cell nevus syndrome

Synonym(s)Nevoid basal cell carcinoma syndrome;Gorlin syndrome; Gorlin-Goltz syndrome;bifid-rib basal-cell nevus syndrome

DefinitionInherited group of defects involving theskin, nervous system, eyes, endocrineglands, and bones, producing an unusualfacial appearance and a predisposition forskin cancers

PathogenesisChromosomal mutation of the PTC gene, atumor suppressor gene; inactivation of thisgene associated with development of basalcell carcinoma, other tumors, and develop-mental errors

Clinical manifestationPitting of the palms or soles; multiple basalcell carcinomas, often early in life; jawcysts; cleft palate; coarse facies with milia,frontal bossing, widened nasal bridge, andmandibular prognathia; strabismus; dys-trophic canthorum; ocular hypertelorism;calcification of the falx cerebri; spine andrib abnormalities; high arched eyebrowsand palate; kidney anomalies; hypogonad-ism in males

Differential diagnosisNon-syndromic basal cell carcinoma; Bazexsyndrome; linear unilateral basal cell nevuswith comedones; Rasmussen syndrome;Rombo syndrome

TherapyMedical therapy: fluorouracil cream;isotretinoin; radiation therapySurgical therapy: primary tumor in ana-tomically insensitive sites – destruction byelectrodesiccation and curettage; ellipticalexcision; cryotherapy; fluorouracil cream;recurrent tumor or those in anatomicallysensitive sites: Mohs micrographic sur-gery�

ReferencesGorlin RJ (1987) Nevoid basal-cell carcinoma syn-

drome. Medicine 66(2):98–113

Basal cell papilloma

� Seborrheic keratosis

Basalioma


Basan syndrome

Synonym(s)Ectodermal dysplasia absent der-matoglyphics

DefinitionAutosomal dominant syndrome consistingof ectodermal dysplasia, absent derma-toglyphic pattern, nail abnormalities, and asimian crease

PathogenesisInherited; mutation site unknown


88 Bather’s itch

Clinical manifestationThin skin; simian crease; multiple dentalcaries; absent or decreased eyebrows; naildystrophy; sparse or absent scalp hair;decreased sweating; photophobia; absentdermatoglyphic pattern

Differential diagnosisAnhidrotic ectodermal dysplasia; hidroticectodermal dysplasia; focal dermal hypo-plasia; Down’s syndrome; progeria


ReferencesMasse JF, Perusse R (1994) Ectodermal dysplasia.

Archives of Disease in Childhood 71(1):1–2

Bather’s itch

� Cercarial dermatitis

Bazin’s disease

� Nodular vasculitis

Beals’ arachnodactyly

� Beals-Hecht syndrome

Beals’ syndrome


Beals-Hecht syndrome

Synonym(s)Beals’ arachnodactyly; Beals’ syndrome;Hecht-Beals syndrome; congenital contrac-tural arachnodactyly syndrome

DefinitionHeritable disorder of connective tissue,present from birth, combining features ofMarfan’s syndrome with arthrogryposis

PathogenesisUnknown; autosomal dominant inheritance

Clinical manifestationMultiple, congenital, joint contractures;arachnodactyly; dolichostenomelia; kypho-scoliosis; changes of the ear muscle, pro-ducing crumpled-appearing ears

Differential diagnosisMarfan’s syndrome; Stickler’s syndrome

TherapyNone

ReferencesJones JL, Lane JE, Logan JJ, Vanegas ME (2002)

Beals-Hecht syndrome. Southern Medical Jour-nal 95(7):753–755

Bean syndrome

� Blue rubber bleb nevus syndrome

Bean-Walsh angioma

� Venous lake


Beckwith-Wiedemann syndrome 89

BBeau’s lines

DefinitionTransverse grooves or lines seen on finger-nails following systemic illness, localtrauma, or skin disease involving the fin-gertips

ReferencesDe Berker D (1994) What do Beau's lines mean?

International Journal of Dermatology 33(8):545–546

Becker melanosis

� Becker’s nevus

Becker nevus


Becker pigmented hairy nevus


Becker’s nevus

Synonym(s)Becker melanosis; Becker nevus; Becker’spigmented hairy nevus; Becker pigmentedhairy nevus; nevus spilus tardus; pigmentedhairy epidermal nevus

DefinitionAcquired melanosis and hypertrichosis in aunilateral distribution

PathogenesisAndrogens possibly a factor in growth ofthe lesion

Clinical manifestationAsymptomatic, irregular, tan-to-brownpatch, most commonly located over thechest, shoulder, or back; often at the time ofpuberty; thick, brown-to-black hairs devel-ops both within and in close proximity tothe patch; possibly associated with underly-ing smooth muscle hamartoma

Differential diagnosisMelanoma; café au lait macule; Albright’ssyndrome; congenital melanocytic nevus;nevus spilus; postinflammatory hyperpig-mentation

TherapyTreatment for cosmetic reasons only – sur-gical excision; Q-switched ruby laser abla-tion; Q-switched neodymium: yttrium-alu-minium-garnet (YAG) laser

ReferencesGoldman MP, Fitzpatrick RE (1994) Treatment of

benign pigmented cutaneous lesions. Cutane-ous Laser Surgery 106–141

Becker’s pigmented hairy nevus


Beckwith-Wiedemann syndrome

Synonym(s)None

DefinitionDisorder consisting of macroglossia, viscer-omegaly, large body size, umbilical herniaor omphalocele, neonatal hypoglycemia


90 Bed sore

PathogenesisSometimes occurring with chromosome 11defect

Clinical manifestationLarge at birth; abdominal wall defect, suchas an umbilical hernia or omphalocele; dis-tinctive facial appearance with a gapingmouth and large tongue; increased inci-dence of childhood tumors, such as Wilmstumor or adrenal carcinoma

Differential diagnosisChildren presenting with overgrowth:Simpson-Golabi-Behmel syndrome; Perl-man syndrome; Costello syndrome; pro-teus syndrome; Klippel-Trenaunay-Webersyndrome; neurofibromatosis

TherapyNeonatal hypoglycemia: intravenous glu-cose; defects of the abdominal wall: surgi-cal repair

ReferencesWeng EY, Mortier GR, Graham JM Jr (1995) Beck-

with-Wiedemann syndrome. An update and review for the primary pediatrician. Clinical Pediatrics 34(6):317–326

Bed sore

� Decubitus ulcer

Bednar tumor

� Dermatofibrosarcoma protuberans

Bee sting

� Hymenoptera sting

Behçet disease

� Behçet’s disease

Behçet’s disease

Synonym(s)Behçet disease; Behçet’s syndrome

DefinitionChronic, inflammatory disorder of bloodvessels, resulting in recurrent oral ulcers,genital ulcers, eye inflammation, and inter-nal organ involvement

PathogenesisUnknown; immune reactions involvingblood vessels cause many of the signs andsymptoms

Clinical manifestationMucocutaneous lesions: erythema nodo-sum; subcutaneous thrombophlebitis; fol-liculitis; acne-like lesions; cutaneous hyper-sensitivity (pathergy); recurrent oral andgenital aphthaeEye lesions: anterior or posterior uveitis;chorioretinitis; arthritis without deformityor ankylosisGastrointestinal lesions: ileocecal ulcers;epididymitis; central nervous system symp-toms

Differential diagnosisAphthous stomatitis; pemphigus vulgaris;herpes simplex virus infection; lichen pla-nus; acute neutrophilic dermatosis; inflam-matory bowel disease; Stevens-Johnsonsyndrome; lupus erythematosus

TherapyLocal therapy: tetracycline suspension(250 mg capsule contents suspended in 5 mlof water) applied to mouth or genital ulcers4 times daily; high potency topical corticos-teroid gel; Kaopectate applied to ulcer 3–4


Benign chronic T-cell infiltrative disorder 91

B

times per day; Zilactin gel applied 4–5 timesper day; viscous lidocaine applied asneeded; amlexanox 5% paste applied 4times dailySystemic therapy: thalidomide; prednisone;azathioprine; cyclosporine; colchicine

ReferencesLee LA (2001) Behcet disease. Seminars in Cuta-

neous Medicine & Surgery 20(1):53–57

Behçet’s syndrome

� Behçet’s disease

Bejel

Synonym(s)Non-venereal syphilis of children; endemicsyphilis

DefinitionNon-venereal disease caused by Treponemaendemicum, transmitted chiefly by directcontact, among children living in tropicaland subtropical climates

PathogenesisOrganism invades through traumatizedcutaneous or mucosal surfaces that come incontact with a draining open sore of theindex case; subsequent spread from origi-nal site either locally by scratching or by thehematogenous route

Clinical manifestationPrimary stage: painless ulcers within theoral cavity; sometimes also appearing as anipple ulceration of a mother with a suck-ling infected childSecondary stage: eroded plaques on thelips, tongue, and tonsils; angular stomatitisvitamin B deficiency; condyloma lata-like

lesions in the anogenital area; generalizedlymphadenopathy; painful osteoperiostitisin the long bonesTertiary (late) stage: gummas which destroybone and cartilage, particularly of the nose,causing saddle nose deformity

Differential diagnosisSyphilis; yaws; pinta; atopic dermatitis; der-matophytosis; psoriasis; leprosy; herpessimplex virus infection; perlèche; condylo-mata acuminata; lupus vulgaris; lupus ery-thematosus; squamous cell carcinoma

TherapyPenicillin G benzathine�; tetracycline;erythromycin

ReferencesKoff AB, Rosen T (1993) Nonvenereal treponema-

toses: yaws, endemic syphilis, and pinta. Jour-nal of the American Academy of Dermatology 29(4):519–535

Benign calcifying epithelioma

� Pilomatricoma

Benign calcifying epithelioma of Malherbe

� Pilomatricoma

Benign chronic T-cell infiltrative disorder

� Jessner lymphocytic infiltration of skin


92 Benign lichenoid keratosis

Benign lichenoid keratosis

� Lichenoid keratosis

Benign lymphoreticulosis

� Bartonellosis

Benign migratory glossitis

Synonym(s)Geographic tongue; stomatitis areatamigrans, erythema areata migrans

DefinitionMap-like appearance of the tongue result-ing from irregular migratory denudedplaques on its surface

PathogenesisUnknown; results from the loss of papillaeof tongue, giving areas of the tongue a flatsurface, and the subsequent geographicappearance; may be related to local traumaor irritants

Clinical manifestationIrregular, smooth, red plaques on the dor-sal surface of the tongue, rapidly changingin pattern; surrounding the area of ery-thema and loss of filiform papillae is a well-defined hyperkeratotic yellow-white borderwith an irregular outline; often associatedwith burning sensation

Differential diagnosisLingua plicata; contact stomatitis; candidia-sis; psoriasis; lichen planus

TherapyNo therapy indicated

ReferencesDelaney JE (1995) Periodontal and soft-tissue ab-

normalities. Dental Clinics of North America 39(4):837–850

Benign mixed tumor of melanocytes and malpighian cells

� Melanoacanthoma

Benign mucous membrane pemphigoid

� Cicatricial pemphigoid

Benign nerve sheath tumor

� Neurothekeoma

Benign papillomatosis of nipple


Benign parapsoriasis

� Small plaque parapsoriasis


Benign symmetric lipomatosis 93

BBenign pigmented purpura

Synonym(s)Pigmented purpuric dermatitis; pigmentedpurpuric eruption; subgroups: Schambergdisease (progressive pigmentary dermato-sis); itching purpura of Loewenthal;eczematid-like purpura of Doucas andKapetanakis; pigmented purpuric lichenoiddermatosis of Gougerot and Blum; lichenaureus; purpura annularis telangiectoides(Majocchi disease)

DefinitionGroup of chronic diseases characterized byextravasation of erythrocytes in the skinwith marked hemosiderin deposition

PathogenesisVenous hypertension, exercise, and gravita-tional dependency possible cofactors

Clinical manifestationReddish-brown, speckled discoloration inpatches or plaquesSchamberg variant: cayenne pepper-likepunctate petechial macules in a larger pur-puric patchLichen aureus variant: golden-yellow patch,most commonly on the legMajocchi variant: annular patches of pur-pura with telangiectasiaGougerot and Blum variant: lichenoid sur-face change

Differential diagnosisThrombocytopenia; cryoglobulinemia;cutaneous T-cell lymphoma; clotting disor-ders; stasis pigmentation; scurvy; leuko-cytoclastic vasculitis; drug hypersensitivityreaction

TherapyTopical corticosteroid, mid potency

ReferencesPiette WW (1994) The differential diagnosis of

purpura from a morphologic perspective. Ad-vances in Dermatology 9:3–23

Benign schwannoma

� Neurilemmoma

Benign symmetric lipomatosis

Synonym(s)Madelung’s disease; cervical lipomatosis;Launois-Bensaude syndrome; multiplesymmetrical lipomatosis; horse-collar neck

DefinitionProgressive, symmetric deposition of adi-pose tissue around the postauricular area,neck, and shoulders

PathogenesisSympathetic denervation locally may be anetiologic factor

Clinical manifestationDiffuse and symmetrical fat deposition in a“horse-collar” distribution around theneck; occasional fat deposition at other sites

Differential diagnosisObesity; Dercum’s disease; multiple heredi-tary lipomatosis; lymphadenopathy; softtissue neoplasms

TherapyLiposuction; surgical excision

ReferencesRuzicka T, Vieluf D, Landthaler M, Braun-Falco O

(1987) Benign symmetric lipomatosis Launois-


94 Benzoyl peroxide

Bensaude. Report of ten cases and review of the literature. Journal of the American Academy of Dermatology 17(4):663–674

Benzoyl peroxide

Trade name(s)Benoxyl; Benzac AC; Benza-Gel; Brevoxyl;Desquam-E; PanOxyl; Persa-Gel; Triaz;combination benzoyl peroxide products:Benzamycin; BenzaClin; Duac


Drug classAntibiotic

Mechanism of actionFree-radical, oxygen-mediated bacterio-cidal effects on P. acnes in sebaceous folli-cles

Dosage form2.5 %, 4%, 5%, 8%, 10% cream, gel, lotion


Common side effectsCutaneous: dryness, erythema, peeling,contact dermatitis


Drug interactionsIsotretinoin

Other interactionsFabrics: may cause color bleaching


ReferencesBasak PY, Gultekin F, Kilinc I, Delibas N (2002)

The effect of benzoyl peroxide and benzoyl per-oxide/erythromycin combination on the anti-oxidative defence system in papulopustular acne. European Journal of Dermatology 12(1):53–57

Bequez Cesar syndrome

� Chédiak-Higashi syndrome

Berardinelli syndrome

� Berardinelli-Seip syndrome


Synonym(s)Berardinelli syndrome; Berardinelli-Seip-Lawrence syndrome; Miescher syndrome 2;Seip syndrome; generalized lipodystrophy

DefinitionAcquired complex of acanthosis nigricans,generalized lipodystrophy, diabetes melli-tus, and hyperlipemia

PathogenesisMonogenic defect, type unknown

Benzoyl peroxide. Dermatologic indications and dosage


Acne vulgaris Apply twice per day Apply twice per day


Berloque dermatitis 95

B

Clinical manifestationOften preceded by an illness; absence of fatclinically evident by age 15 years; acantho-sis nigricans; diabetes mellitus; associatedautoimmune disorders; prone to infection

Differential diagnosisLawrence-Seip syndrome; progressive par-tial lipodystrophy; post-traumatic partiallipodystrophy

TherapyDietary fish oil supplementation; acitretin

ReferencesSeip M, Trygstad O (1996) Generalized lipodys-

trophy, congenital and acquired (lipoatrophy). Acta Paediatrica Suppl413:2

Berardinelli-Seip-Lawrence syndrome


Bergamot phototoxicity

� Berloque dermatitis

Bergapten phototoxicity


Berkshire neck

� Poikiloderma of Civatte

Berlock dermatitis


Berloque dermatitis

Synonym(s)Berlock dermatitis; perfume phototoxicity;bergapten phototoxicity; bergamot photo-toxicity; photodermatitis pigmentaria;dermite pigmentée en forme de coulée

DefinitionPhototoxic reaction induced by the effect oflong-wave ultraviolet (UVA) radiation onbergapten (5-methoxypsoralen), a photoac-tive component of bergamot oil

PathogenesisPhotoactivation of bergapten by UVA radia-tion, causing phototoxicity and melanocytestimulation to produce melanin; distribu-tion of melanosomes in keratinocytechanging from the aggregate to disaggre-gated form, similar to that seen in skin ofblack individuals

Clinical manifestationErythema; edema; vesiculation; desquama-tion; pendant-like hyperpigmentation atsites of oil of bergamot application, oftenon the lateral neck

Differential diagnosisContact dermatitis; Riehl melanosis;melasma; postinflammatory hyperpigmen-tation; acanthosis nigricans

TherapyAvoidance of bergamot oil-containing per-fumes; minimized exposure to the sun(sunscreens, etc.); hydroquinone


96 Besnier-Boeck-Schaumann disease

ReferencesNone

Besnier-Boeck-Schaumann disease

� Sarcoidosis

Besnier's prurigo


Betamethasone

� Corticosteroids, topical, medium potency

Betamethasone dipropionate


Betamethasone valerate


Beurmann's disease

� Sporotrichosis

Bifid-rib basal-cell nevus syndrome

� Basal cell nevus syndrome

Biskra button


Black bane

� Anthrax, cutaneous

Black blood


Black dot ringworm

DefinitionAppearance of punctate black dots repre-senting broken hairs at sites of tinea capi-tis, caused by the fungal pathogen, T. ton-surans

ReferencesElewski BE (2000) Tinea capitis: A current per-

spective. Journal of the American Academy of Dermatology 42(1 Pt 1):1–20

Black hairy tongue

� Hairy tongue


Blistering distal dactylitis 97

BBlack heel

Synonym(s)Talon noir; tennis heel; hyperkeratosishaemorrhagica; pseudochromhidrosisplantaris; calcaneal petechiae

DefinitionSelf-limited, asymptomatic, trauma-induced darkening of the posterior or pos-terolateral aspect of the heel occurring afterminor trauma, mostly from athletic pur-suits

PathogenesisLateral shearing force of the epidermis slid-ing over the rete pegs of the papillary der-mis, resulting in hemorrhage

Clinical manifestationMultiple asymptomatic petechiae centrallyaggregated with a few scattered satellitepatches, located over posterior and postero-lateral heel

Differential diagnosisMelanoma; wart; nevus; lentigo

TherapyParing of lesion with a scalpel blade; protec-tive heel pad for prophylaxis

ReferencesLevine N, Baron J (2000) Black Heel in: James

WD, Elston D (Chief eds.) eMedicine Derma-tology St. Petersburg: eMedicine Corporation

Black piedra

� Piedra

Blaschkitis

� Lichen striatus

Blaschko linear acquired inflammatory skin eruption

� Lichen striatus

Blastomycosis

� North American blastomycosis

Blennorrheal idiopathic arthritis

� Reiter syndrome

Blepharochalasis

� Dermatochalasis

Blinding filariasis

� Filariasis

Blistering dactylitis

� Blistering distal dactylitis

Blistering distal dactylitis

Synonym(s)Blistering dactylitis


98 Bloch-Siemens syndrome

DefinitionSuperficial infection of the anterior fat padof distal phalanx, usually caused by β-hemolytic streptococcal pathogens

PathogenesisS. pyogenes colonizes normal skin surfacesfor extended periods; following acquisitionon the normal skin, minor trauma may be aprerequisite for initiating infection

Clinical manifestationTender vesicle or bulla on an erythematousbase, covering the volar surface of theaffected digit

Differential diagnosisHerpetic whitlow; friction blister; epider-molysis bullosa; burn trauma

TherapyPenicillin G benzathine; penicillin VK; inci-sion and drainage

ReferencesNey AC, English JC 3rd, Greer KE (2002) Coexist-

ent infections on a child's distal phalanx: blis-tering dactylitis and herpetic whitlow. Cutis 69(1):46–48

Bloch-Siemens syndrome

� Incontinentia pigmenti

Bloch-Sulzberger syndrome

� Incontinentia pigmenti

Bloom syndrome

� Bloom’s syndrome

Bloom’s syndrome

Synonym(s)Bloom syndrome; congenital telangiectaticerythema

DefinitionAutosomal recessive disorder characterizedby telangiectases and photosensitivity,growth deficiency, a variable degree ofimmunodeficiency, and increased suscepti-bility to neoplasms

PathogenesisMutation in the gene designated BLM, on15q26.1; protein encoded by the normalgene has DNA helicase activity and func-tions in the maintenance of genomic stabil-ity; mutation likely responsible for the phe-notype and the cancer predisposition

Clinical manifestationTelangiectatic erythema in photodistrib-uted pattern; cheilitis; café au lait macules;bird-like facies; malar hypoplasia, smallmandible; large, protruding ears; growthdelay; short stature; malignancies, such asacute leukemia, lymphoma, and gastroin-testinal adenocarcinoma

Differential diagnosisCockayne syndrome; Rothmund-Thomsonsyndrome; lupus erythematosus; erythro-poietic protoporphyria

TherapyNo specific treatment; sun protection

ReferencesGerman J (1995) Bloom's syndrome. Dermatolog-


Blue neuronevus

� Blue nevus


Boeck's sarcoid 99

BBlue nevus

Synonym(s)Nevus of Jadassohn and Tieche; blue neu-ronevus; dermal melanocytoma

DefinitionBlue or blue-black skin lesion produced bya collection of functioning deep dermalmelanocytes

PathogenesisDermal arrest in fetal migration of melano-cytes of neural crest origin results in failureto reach the epidermis

Clinical manifestationSmooth-surfaced, dome-shaped blue orblue-gray papules; common blue nevi lessthan 1 cm; cellular variant sometimes largerthan 1 cm

Differential diagnosisMelanoma; traumatic tattoo; seborrheickeratosis; dermatofibroma; nevus of Ota/Ito; cherry hemangioma

TherapyDiagnostic biopsy if melanoma seriouslyconsidered; simple excision for cosmeticreasons�

ReferencesSchaffer JV, Bolognia JL (2000) The clinical spec-

trum of pigmented lesions. Clinics in Plastic Surgery 27(3):391–408

Blue rubber bleb nevus syndrome

Synonym(s)Bean syndrome

DefinitionDisorder characterized by multiple cutane-ous venous malformations with visceral

lesions most commonly affecting the gas-trointestinal tract

PathogenesisUnknown

Clinical manifestationMultiple, protuberant, dark blue, compress-ible nodules; large, cavernous lesions thatmay compress vital structures; irregularblue macules or patches; multiple gastroin-testinal hemangiomas which may bleed,cause intussusception, volvulus, or bowelinfarction; other extra-cutaneous sites,including skull, central nervous system,thyroid, parotid gland, eyes, oral cavity,lungs, pleura, pericardium, musculoskele-tal system, peritoneal cavity, mesentery,kidney, liver, spleen, penis, vulva, and blad-der

Differential diagnosisArteriovenous malformation; Kaposi’s sar-coma; Mafucci syndrome; Klippel-Trenau-nay-Weber syndrome

TherapyDestruction by electrodesiccation andcurettage, liquid nitrogen cryotherapy; sur-gical excision; CO2 laser vaporization

ReferencesMoodley M, Ramdial P (1993) Blue rubber bleb

nevus syndrome: case report and review of the literature. Pediatrics 92(1):160–162

Boder-Sedgwick syndrome

� Ataxia-telangiectasia

Boeck's sarcoid

� Sarcoidosis


100 Boil

Boil

� Furuncle

Bonnet-Dechaume-Blanc syndrome

� Wyburn-Mason syndrome

Bonnevie-Ullrich syndrome

� Turner syndrome

Böök syndrome

Synonym(s)Böök’s syndrome

DefinitionSyndrome consisting of premature grayingof the hair, tooth development abnormali-ties, and palmoplantar hyperhidrosis

PathogenesisAutosomal dominant inheritance; unknowngenetic defect

Clinical manifestationGraying of the hair before age 14 years,mostly in the scalp hair; bicuspid aplasia;hyperhidrosis of the palms and soles

Differential diagnosisEctodermal dysplasia; essential hyperhidro-sis

TherapyNone

ReferencesBöök JA (1950) Clinical and genetical studies of

hypodontia. Premolar aplasia, hyperhidrosis, and canities prematura: A new hereditary syn-drome in man. American Journal of Human Genetics 2:240–263

Böök’s syndrome

� Böök syndrome

Boston exanthem

Synonym(s)Echovirus 16 infection

DefinitionSkin eruption caused by an infection withechovirus 16

PathogenesisEchovirus 16 infection acquired by the oralor respiratory route

Clinical manifestationProdrome of fever and anorexia after 3–8day incubation period; exanthem begins onthe face and chest and quickly generalizes;enanthem occasionally present; posteriorauricular lymphadenopathy

Differential diagnosisRoseola; other viral exanthems; meningo-coccal infection; medication reaction

TherapyNone; no specific isolation needed

ReferencesCherry JD (1983) Viral exanthems. Current Prob-

lems in Pediatrics 13(6):1–44


Bowen disease 101

BBotryomycosis

Synonym(s)Granular bacteriosis; actinophytosis

DefinitionChronic, purulent, bacterial infection withformation of granules

PathogenesisMost cases caused by Staphylococcusaureus; may represent suboptimal hostresponse to bacterial organism

Clinical manifestationCutaneous or subcutaneous doughy nod-ules with ulcerations and draining sinuses;grainy material extruding from the lesions

Differential diagnosisMycetoma; actinomycosis; kerion; subcuta-neous granuloma annulare; Kaposi’s sar-coma; lymphoma

TherapySurgical therapy: debridement; surgicalexcision; laser vaporizationMedical therapy: dicloxacillin; cephalexin

ReferencesBonifaz A, Carrasco E (1996) Botryomycosis. In-

ternational Journal of Dermatology 35(6):381–388

Bouba

� Yaws

Bourneville disease

� Tuberous sclerosis

Boutonneuse fever

Synonym(s)Mediterranean spotted fever; Carduccifever; tick typhus; South African ticktyphus; Indian tick typhus; tick bite fever

DefinitionRickettsial disease caused by Rickettsiaconorii occurring in the Mediterraneanbasin

PathogenesisOrganism introduced through a tick bite;invasion and proliferation in the endothe-lial cells of small vessels

Clinical manifestationHistory of recent travel in endemic area;fever; erythematous papules, mainly on thelower extremities; eschar at the site of thetick bite; localized or generalized purpura

Differential diagnosisViral exanthem; Rocky Mountain spottedfever; rubeola; Lyme disease; medicationreaction; Kawasaki disease; aseptic acutearthritis; leukocytoclastic vasculitis

TherapyDoxycycline; ciprofloxacin 250–500 mg POtwice daily for 7–14 days

ReferencesCascio A, Dones P, Romano A (1998) Clinical and

laboratory findings of boutonneuse fever in Si-cilian children. European Journal of Pediatrics 157(6):482–486

Bowen disease

� Bowen’s disease


102 Bowen’s carcinoma

Bowen’s carcinoma


Bowen’s disease

Synonym(s)Squamous cell carcinoma in-situ; Bowendisease; Bowen’s carcinoma

DefinitionCutaneous squamous cell carcinoma withfull thickness dysplasia of the epidermiswithout dermal invasion

PathogenesisChronic solar damage, inorganic arsenicingestion, and certain human papillomavirus subtypes (12, 13, 16 and 34) implicatedas etiologic factors

Clinical manifestationRed, scaly, non-substantive papule orplaque, most often occurring on the headand neck, but sometimes appearing onother sun-exposed areas or the trunk

Differential diagnosisActinic keratosis; superficial basal cell car-cinoma; seborrheic keratosis; lichenoidkeratosis; extramammary Paget’s disease;psoriasis; tinea corporis; lupus erythemato-sus

TherapyDestruction by electrodesiccation andcurettage or liquid nitrogen cryotherapy;surgical excision; fluorouracil cream; pho-todynamic therapy

ReferencesFitzgerald DA (1998) Cancer precursors. Semi-

nars in Cutaneous Medicine & Surgery 17(2):108–113

Bowenoid papulosis

Synonym(s)Viral keratoses; bowenoid papulosis of thepenis; bowenoid papulosis of the genitalia

DefinitionHuman papillomavirus (HPV)-inducedwart with histologic features of Bowen’s dis-ease

PathogenesisHPV type 16 most common causative agent;sometimes also occurring with viral types18, 31, 32, 33, 34, 35, 39, 42, 48, 51, 52, 53, and54

Clinical manifestationSolitary or multiple, pigmented papuleswith a flat-to-velvety surface; lesions some-times coalescing into plaques; occur mostcommonly on the penile shaft or the exter-nal genitalia of females

Differential diagnosisSeborrheic keratosis; squamous cell carci-noma; melanocytic nevus; lichen planus

TherapyLocal excision; destruction by electrodesic-cation and curettage, cryosurgery, CO2laser ablation; tretinoin; podofilox; 5-fluor-ouracil cream

ReferencesSchwartz RA, Janniger CK (1991) Bowenoid papu-

losis. Journal of the American Academy of Der-matology 24(2 Pt 1):261–264

Bowenoid papulosis of the genitalia

� Bowenoid papulosis


Brauer’s syndrome 103

BBowenoid papulosis of the penis


Brachmann-de Lange syndrome


Branchial cleft cyst

Synonym(s)Branchial cyst; lateral cervical cyst;branchiogenic cyst; branchioma

DefinitionEpithelial cyst arising on the lateral neckfrom a failure of obliteration of the secondbranchial cleft during embryonic life

PathogenesisBranchial arch clefts, which normally invo-lute by week 7 of embryonic development,become ectoderm-lined cavities; withincomplete involution, entrapped remnantforms an epithelium-lined cyst

Clinical manifestationAsymptomatic, fluctuant nodule, occurringalong the lower portion of the anterome-dial border of the sternocleidomastoidmuscle between the muscle and overlyingskin; sometimes becomes tender if second-arily inflamed or infected; with a sinustract, occasional mucoid or purulent exu-date

Differential diagnosisLymphadenopathy; vascular malformation;hemangioma; carotid body tumor; cystichygroma; ectopic salivary or thyroid tissue

TherapySurgical excision, usually after age 3months�; incision and drainage if abscessforms

ReferencesBrown RL, Azizkhan RG (1998) Pediatric head

and neck lesions. Pediatric Clinics of North America 45(4):889–905

Branchial cyst

� Branchial cleft cyst

Branchiogenic cyst


Branchioma


Brauer’s syndrome

Synonym(s)Focal facial dysplasia; hereditary symmetri-cal aplastic nevi of the temples

DefinitionHereditary, focal pigmented nevi of theforehead and chin associated with eitherthe absence of eyelashes or double rows ofeyelashes and absence of sweat glands inthe lesions

PathogenesisUnknown; autosomal dominant inheritance


104 Brazilian blastomycosis

Clinical manifestationHereditary, focal pigmented nevi, similar toforceps marks; located on the forehead andchin; absence of eyelashes or double rows ofeyelashes and aplasia of the sweat glands inthe lesions; protuberant nose

Differential diagnosisHypohidrotic ectodermal dysplasia

TherapyNone

ReferencesPinheiro M, Freire-Maia N (1994) Ectodermal

dysplasias: A clinical classification and a causal review. American Journal of Medical Genetics 53(2):153–162

Brazilian blastomycosis

� South American blastomycosis

Brazilian pemphigus

� Fogo selvagem

Brazilian pemphigus foliaceus

� Fogo selvagem

Brill-Zinsser disease

� Typhus

Brocq pseudopelade

� Pseudopelade

Brocq’s disease


Broken capillaries

� Varicose and telangiectatic leg veins

Bromhidrosis

Synonym(s)Apocrine bromhidrosis; osmidrosis;bromidrosis

DefinitionCondition of abnormal or offensive bodyodor

PathogenesisOdor as a consequence of apocrine glandsecretion; bacterial decomposition of apo-crine secretion yields short-chain fattyacids with characteristic odors; other odor-inducing situations include metabolic dis-orders, ingestion of foods or drugs, or toxicmaterials, or contact with certain xenobiot-ics

Clinical manifestationAppearance normal except when associ-ated with other unrelated conditions, suchas erythrasma or intertrigo

Differential diagnosisFish odor syndrome (trimethylaminuria);organic brain lesions (tumors, etc.); bodydysmorphic disorder


Brown recluse spider bite 105

B

TherapyHygienic measures: adequate washing ofthe axillary vault; drying powders; frequentclothing changesDiet: omission of certain foods (e.g. certainspices, garlic, alcohol) in instances whencontributorySurgical: superficial liposuction to removeapocrine glands

ReferencesLockman DS (1981) Olfactory diagnosis. Cutis

27(6):645–647

Bromidrosis

� Bromhidrosis

Bromoderma

� Halogenoderma

Brompheniramine

� Antihistamines, first generation

Bronze baby syndrome

Synonym(s)Brown baby syndrome

DefinitionComplication of neonatal phototherapy ininfants with hepatic disease, with skin tak-ing on a bronze color

PathogenesisProposed mechanisms including photode-struction of porphyrin and deposition in

the skin or a deposition of a photo-isomerof bilirubin the skin

Clinical manifestationDark gray-brown discoloration of the entireskin surface, fading approximately 6 weeksafter stopping phototherapy

Differential diagnosisGray baby syndrome (chloramphenicoloverdosage); cyanosis

TherapyEvaluation and treatment of underlyinghyperbilirubinemia

ReferencesRubaltelli FF, Da Riol R, D'Amore ES, Jori G (1996)

The bronze baby syndrome: evidence of in-creased tissue concentration of copper porphy-rins. Acta Paediatrica 85(3):381–384

Bronze diabetes

� Hemochromatosis

Brooke tumor

� Trichoepithelioma

Brown baby syndrome

� Bronze baby syndrome

Brown recluse spider bite

Synonym(s)Necrotic arachnidism; arachnidism;loxoscelism; latrodectism


106 Brown spot syndrome

DefinitionSkin necrosis and sloughing secondary tothe bite of the brown recluse spider

PathogenesisEnvenomation from brown recluse spider(Loxosceles reclusa); phospholipase D maintoxic factor

Clinical manifestationBite minimally symptomatic; fewer than10% of bites result in severe skin necrosis;signs of progression within 48–72 hours ofthe bite; mild-to-severe pain beginning 2–8hours after bite; central papule and associ-ated erythema occur 6–12 hours after bite;purple vesicle sometimes ulcerates; stellatenecrotic area sometimes ensues.Constitutional signs and symptoms: hemol-ysis; hemoglobinuria; thrombocytopenia;disseminated intravascular coagulation;fever; headache; malaise; arthralgia; nau-sea; vomiting

Differential diagnosisPyoderma gangrenosum; ecthyma; herpessimplex virus infection; insect bite reac-tion; squamous cell carcinoma; coumarinnecrosis; vasculitis; vascular insufficiency;necrotizing fasciitis; factitial ulceration;thromboembolic phenomenon; skintrauma; thromboangiitis obliterans; neuro-pathic ulceration; tularemia; mucormycosis

TherapyLocal therapy: cleansing of the bite site;cold compresses; simple analgesics; eleva-tion of an affected extremity; intralesionalcorticosteroidsSystemic therapy: dapsone; prednisone forsystemic signs and symptomsSurgical therapy: excision of necrotic areaonly after 6 weeks if healing not progress-ing

ReferencesSams HH, Dunnick CA, Smith ML, King LE Jr

(2001) Necrotic arachnidism. Journal of the American Academy of Dermatology 44(4):561–573

Brown spot syndrome


Brugian filariasis

� Filariasis

Brunaur-Fuhs-Siemens syndrome

� Striate keratoderma

Bulldog scalp

� Cutis verticis gyrata

Bullous congenital ichthyosiform erythroderma

� Epidermolytic hyperkeratosis

Brown recluse spider bite. Plaque with early necrosis in the center and an erythematous border,,


Bullous pemphigoid 107

BBullous diabeticorum

� Bullous eruption of diabetes mellitus

Bullous disease of diabetes mellitus


Bullous eruption of diabetes mellitus

Synonym(s)Bullous disease of diabetes mellitus; bullousdiabeticorum; diabetic bullae

DefinitionNon-inflammatory, blistering condition ofacral skin in patients with diabetes mellitus

PathogenesisPossibly related to diabetic neuropathy ornephropathy; possibly associated withdefect in anchoring fibrils

Clinical manifestationNon-inflammatory vesicles and bullae,most commonly on the hands and lowerlegs

Differential diagnosisBullous pemphigoid; epidermolysis bullosaacquisita; porphyria cutanea tarda; burn;friction blister; blistering distal dactylitis


ReferencesBasarab T, Munn SE, McGrath J, Russell Jones R

(1995) Bullosis diabeticorum. A case report and

literature review. Clinical & Experimental Der-matology 20(3):218–220

Bullous ichthyosiform erythroderma


Bullous ichthyotic erythroderma


Bullous pemphigoid

Synonym(s)Pemphigoid; pemphigoid vegetans

DefinitionAutoimmune, blistering disease character-ized by the presence of IgG autoantibodiesspecific for the hemi-desmosomal antigens

Bullous pemphigoid. Numerous tense vesicles and bullae, many of which arise from normal-appearing skin


108 Bullous photosensitivity, drug- or therapy-induced

PathogenesisIgG autoantibodies specific for the hemi-desmosomal bullous pemphigoid antigensBP230 (BPAg1) and BP180 (BPAg2); bindingof antibodies at the basement membrane,activating complement and inflammatorymediators and producing injury at the base-ment membrane zone

Clinical manifestationTense vesicles and bullae, with a predilec-tion for the flexor areas of the skin; oral andocular mucosa involvement seldom occurs;bullae clinically either inflammatory ornon-inflammatory; blisters usually healwithout scarring or milia formation; local-ized form with blisters confined to theextremities; lesions sometimes urticarialwithout vesiculation

Differential diagnosisCicatricial pemphigoid; herpes gestationis;linear IgA bullous dermatosis; dermatitisherpetiformis; chronic bullous dermatosisof childhood; dyshidrosis; bullous lupuserythematosus; pemphigus vegetans; urti-caria

TherapyMild to moderate disease: high potencytopical corticosteroids; combination of tet-racycline and niacinamide 500 mg PO 2-3times dailySevere disease: prednisone; steroid-sparingmedications: azathioprine; cyclophospha-mide; mycophenolate mofetil; dapsone;methotrexate

ReferencesKhumalo NP, Murrell DF, Wojnarowska F, Kirt-

schig G (2002) A systematic review of treat-ments for bullous pemphigoid. Archives of Dermatology 138(3):385–389

Bullous photosensitivity, drug- or therapy-induced

� Pseudoporphyria


Synonym(s)None

DefinitionSensation of burning or pain in the mouthwithout an identifiable visible pathologicprocess responsible for the symptom

PathogenesisPossible etiologic factors: nutritional defi-ciency (e.g. B vitamin deficiency), majordepression; increased taste sensation; men-opause (90% of affected women postmeno-pausal); trigeminal nerve neuropathy

Clinical manifestationBurning pain affecting oropharynx; onsetin the morning; peak symptoms in the lateafternoon; lower lip mucosa, anteriortongue, anterior hard palate affected; painrelief with eating; associated with drymouth and taste disturbance; no evidentlesions

Differential diagnosisTobacco abuse; atrophic glossitis; menopau-sal glossitis; heavy metal poisoning; vita-min deficiency; leukemia; lichen planus;uremia; medication reaction

TherapyCapsaicin: starting with hot pepper diluted1:2 with water; rinsing of mouth with 1 tea-spoon; decreasing dilution to 1:1 as toler-ated; amitriptyline; gabapentin: startingwith 300 mg PO at bedtime, titrating up toa maximum of 1800 mg per day; serotoninreuptake inhibitor

ReferencesMuzyka BC, De Rossi SS (1999) A review of burn-

ing mouth syndrome. Cutis 64(1):29–35


Busse-Buschke disease 109

BBuruli ulcer

Synonym(s)Mycobacterium ulcerans infection

DefinitionChronic, necrotizing disease of the skin dueto Mycobacterium ulcerans

PathogenesisInoculation of Mycobacterium ulcerans intothe skin occurring via trauma; organismproduces mycolactone, an immunosuppres-sive, soluble, polyketide toxin with cyto-toxic properties

Clinical manifestationPresenting as firm, nontender subcutane-ous nodule; within the next 1 to 2 months,area becomes fluctuant and forms a pain-less, undermined ulceration; lesions with ascalloped border and a sloughing, necroticbase; spontaneous healing after manymonths

Differential diagnosisTropical phagedenic ulcer; cutaneous tuber-culosis; deep fungal infection; leishmania-sis; pyoderma gangrenosum; squamous cellcarcinoma; vasculitis

TherapySurgical therapy: excision of ulcer�

Medical therapy: rifampin 600 mg PO perday

Referencesvan der Werf TS, van der Graaf WT, Tappero JW,

Asiedu K (1999) Mycobacterium ulcerans in-fection. Lancet 354(9183):1013–1018

Buschke-Löwenstein tumor

� Giant condyloma of Buschke and Löwenstein

Buschke-Ollendorff syndrome

Synonym(s)Dermatofibrosis lenticularis; disseminatedlenticular dermatofibrosis; dermatofibrosislenticularis disseminata with osteopoikilo-sis

DefinitionEctodermal dysplasia of connective tissue,consisting of osteopoikilosis and connec-tive tissue nevi

PathogenesisPossibly resulting from abnormal regula-tion of extracellular matrix, leading toincreased accumulation of elastin in thedermis

Clinical manifestationAsymptomatic, slightly elevated, yellowishpapules and nodules coalescing to formplaques; arising over several years; oste-opoikilosis of the epiphysis and the meta-physis of long bones

Differential diagnosisPseudoxanthoma elasticum; tuberous scle-rosis; connective tissue nevus; morphea

TherapySurgical excision of skin lesions for cos-metic purposes

ReferencesWoodrow SL, Pope FM, Handfield-Jones SE

(2001) The Buschke-Ollendorff syndrome pre-senting as familial elastic tissue naevi. British Journal of Dermatology 144(4):890–893

Busse-Buschke disease

� Cryptococcosis


C

Café au lait macule

Synonym(s)

Café au lait spot

;

hypermelanotic macule

Definition

Discrete, tan-brown macule with irregularmargins

Pathogenesis

Possibly represents increased melanocytesize or increased melanosome production


Asymptomatic, 2–20 mm discrete tan-brown macule or patch; occurring inpatients with neurofibromatosis, McCune-Albright syndrome, Watson’s syndrome,proteus syndrome, Bloom’s syndrome, pie-baldism, and Fanconi’s anemia


Lentigo; seborrheic keratosis; nevocellularnevus; nevus spilus; multiple lentiginessyndrome

Therapy

Q-switched Nd:YAG or Q-switched rubylaser ablation; hydroquinone

References

Landau M, Krafchik BR (1999) The diagnostic val-ue of cafe-au-lait macules. Journal of the Amer-ican Academy of Dermatology 40(6 Pt 1):877–890

Café au lait spot

�

Café au lait macule

Café-au-lait spots syndrome

�

Watson syndrome

Calcaneal petechiae

�

Black heel

Café au lait macule.

Tan-brown patch on the chest wall

PART3.MIF Friday, October 31, 2003 9:37 AM

112 Calcific uremic arteriolopathy

Calcific uremic arteriolopathy

�

Calciphylaxis

Calcifying epithelioma of Malherbe

�

Pilomatricoma

Calcinosis cutis

Synonym(s)

Cutaneous calcinosis

;

cutaneous calculi

Definition

A group of disorders in which calcium salts,consisting primarily of hydroxyapatite crys-tals or amorphous calcium phosphate, aredeposited in the skin

Pathogenesis

Unclear; involves both metabolic and physi-cal factors; in the setting of hypercalcemiaand/or hyperphosphatemia, calcium depo-sition occurring without preceding tissuedamage; damaged tissue possibly allows aninflux of calcium ions, leading to calciumprecipitation


Multiple, asymptomatic, firm, whitishpapules, plaques, or nodules in the dermisand/or subcutis; sometimes spontaneouslyulcerating and extruding a chalky whitematerial; dystrophic calcinosis cutis: depos-its at the site of trauma; metastatic calcifica-tion: widespread calcinosis, often aroundlarge joints; common in children with der-matomyositis and in those with CREST syn-drome


Gouty tophus; granuloma annulare; xan-thoma; foreign body granuloma; milium;osteoma cutis

Therapy

Surgical excision; sodium etidronate anddiphosphonates; colchicine; warfarin; int-ralesional triamcinolone 3–4 mg per ml

References

Rodriguez-Cano L, Garcia-Patos V, Creus M. Bast-ida P, Ortega JJ, Castells A (1996) Childhood calcinosis cutis. Pediatric Dermatology 13(2):114–117

Calciphylaxis

Synonym(s)

Necrotizing livedo reticularis

;

uremic gan-grene syndrome

;

uremic necrosis

;

calcificuremic arteriolopathy

;

obliterative calcific-thrombotic arteriolopathy

Definition

Syndrome of vascular calcification withcutaneous necrosis, usually in patients withrenal failure

Pathogenesis

Pathogenic factors possibly include chronicrenal failure, hypercalcemia, hyperphos-phatemia, an elevated calcium-phosphateproduct, and secondary hyperparathy-roidism


Presents as livedo reticularis or as ery-thematous papules or plaques, mostly onthe lower extremities, evolving into stellatepurpuric ulcerations with central necrosis;extreme pain and tenderness in lesions


Polyarteritis nodosa; pyoderma gangreno-sum; Wegener’s granulomatosis; lupus ery-thematosus; cryoglobulinemia; coumarin


Calcipotriene 113

C

necrosis; protein C or protein S deficiency;antiphospholipid syndrome; atheroscle-rotic peripheral vascular disease; pancre-atic panniculitis; cholesterol emboli; dis-seminated intravascular coagulation

Therapy

Total or subtotal parathyroidectomy onlywith evidence of hyperparathyroidism;aggressive wound care and debridement ofnecrotic tissue; dietary alteration withphosphate binders and low calcium-bathdialysis to decrease serum calcium andphosphate concentrations

References

Oh DH, Eulau D, Tokugawa DA, McGuire JS, Koh-ler S (1999) Five cases of calciphylaxis and a re-view of the literature. Journal of the American Academy of Dermatology 40(6 Pt 1):979–987

Calcipotriene

Trade name(s)

Dovonex

Generic available

No

Drug class

Vitamin D3 derivative

Mechanism of action

Acts through the vitamin D nuclear recep-tor to regulate growth, differentiation, andimmune functions

Dosage form

0.005% cream, ointment, lotion


See table

Common side effects

Cutaneous:

pruritus; irritant contact der-matitis; erythema


Hematologic:

hypercalcemia

Drug interactions

None



References

Lebwohl M, Ali S (2001) Treatment of psoriasis.Part 1. Topical therapy and phototherapy. Jour-nal of the American Academy of Dermatology45(4):487–98

Calcipotriene. Dermatologic indications and dosage




Grover’s disease Apply twice daily Apply twice daily

Inflammatory linear verrucous nevus


Morphea Apply twice daily Apply twice daily

Porokeratosis Apply twice daily Apply twice daily

Psoriasis Apply twice daily Apply twice daily

Reiter syndrome Apply twice daily Apply twice daily

Vitiligo Apply twice daily Apply twice daily


114 Callosity

Callosity

�

Clavus

Callous

�

Clavus

Callus

�

Clavus

Campbell de Morgan spots

�

Cherry hemangioma

Candidiasis

Synonym(s)

Moniliasis

;

candidosis

;

thrush

Definition

Skin and mucous membrane infectionscaused by Candida species

Pathogenesis

Warm, moist parts of the body susceptibleto infection; host factors such as oralhygiene important in intraoral infection(thrush); primary immune defects in muco-cutaneous candidiasis; endocrinopathiessuch as diabetes mellitus, Cushing’s syn-drome, Addison’s disease, hypoparathy-roidism associated with recurrent infec-tions


Thrush: discrete or confluent white plaqueson oral mucosa common on the tongue;erythema and fissures at the corners of themouthCandida intertrigo: red macerated intertrig-inous areas with satellite pustulesVulvovaginitis: pruritic, white, cheesy dis-charge; beefy red vulvaChronic mucocutaneous variant: whiteadherent plaques of thrush or the angularcheilitis of perlèche; oral involvement mayextend to the esophagus; nails are thick-ened, fragmented, and discolored, with sig-nificant edema and erythema of the sur-rounding periungual tissue; skin lesionsoften are acral or in the scalp, with ery-thematous, hyperkeratotic, serpiginousplaquesInterdigital involvement (erosio interdigi-talis blastomycetica): erythema, scale, andsatellite papules and pustules, most com-monly in the space between the long fingerand ring finger


Thrush: Fordyce spots; hairy leukoplakia;lichen planus; aphthous stomatitis; pemphi-gus vulgaris; herpes simplex virus infectionCandida intertrigo : tinea cruris; contactdermatitis; seborrheic dermatitisInverse psoriasis mucocutaneous variant:acrodermatitis enteropathica; immunodefi-ciency diseases such as HIV infection,DiGeorge syndrome, Nezelof syndrome or

Candidiasis.

Red, fissured plaque at the corner of the mouth


Cantharidin 115

C

severe combined immunodeficiency; nutri-tional deficiency

Therapy

Thrush: clotrimazole 10 mg troche taken 3times daily for 7–10 days; fluconazole; itra-conazoleCandida intertrigo: fluconazole; azole anti-fungal agents; Zeasorb AF powder usedtwice daily as prophylaxisMucocutaneous variant: fluconazole; clot-rimazole 10 mg troche taken 3 times dailyfor 7–10 days; cimetidine 400 mg PO 4times daily indefinitely

References

Zuber TJ, Baddam K (2001) Superficial fungal in-fection of the skin. Where and how it appears help determine therapy. Postgraduate Medi-cine 109(1):117–120,123–126,131–132

Candidosis

�

Candidiasis

Cane-cutter fever

�

Leptospirosis

Canicola fever

�

Leptospirosis

Canities

Definition

Graying or whitening of hair

References

Tobin DJ, Paus R (2001) Graying: gerontobiology of the hair follicle pigmentary unit. Experi-mental Gerontology 36(1):29–54

Canker sore

�

Aphthous stomatitis

Cannon’s disease

�

White sponge nevus

Cantharidin

Trade name(s)

Canthacur

Generic available

No

Drug class

Vesicating agent

Mechanism of action

Interferes with mitochondria, which leadsto epidermal cell necrosis

Dosage form

Colloidal solution


See table

Common side effects

Cutaneous:

irritant dermatitis, pain at siteof application


116 Cantu syndrome


None

Drug interactions

None



References

Moed L, Shwayder TA, Chang MW (2001) Can-tharidin revisited: A blistering defense of an ancient medicine. Archives of Dermatology 137(10):1357–1360

Cantu syndrome

Synonym(s)

Hypertrichotic osteochondrodysplasia

Definition

Hereditary syndrome consisting of congen-ital hypertrichosis; osteochondrodysplasialeading to multiple skeletal defects and car-diomegaly

Pathogenesis

Unknown


Congenital hypertrichosis; macrosomia atbirth; narrow thorax; cardiomegaly; wideribs; hypoplastic ischiopubic branches;small obturator foramen; bilateral coxavalga; enlarged medullary canal; Erlen-meyer-flask-like long bones; generalizedosteopenia


Costello syndrome

Therapy

None

References

Lazalde B, Sanchez-Urbina R, Nuno-Arana I, Bitar WE, de Lourdes Ramirez-Duenas M (2000) Autosomal dominant inheritance in Cantu syndrome (congenital hypertrichosis, osteochondrodysplasia, and cardiomegaly). American Journal of Medical Genetics 94(5):421–427

Capillary angioma

�

Capillary hemangioma


Synonym(s)

Strawberry hemangioma

;

strawberry mark

;

raspberry lesion

;

infantile hemangioma

;

capillary angioma

Definition

Benign vascular neoplasm, consisting ofproliferating endothelial cells, with earlyproliferation, usually followed by spontane-ous involution

Pathogenesis

May involve abnormal release of ang-iogenic factors; possible role of estrogens

Cantharidin. Dermatologic indications and dosage


Molluscum contagiosum

Apply once under tape occlusion for 24 hours


Wart Apply once under tape occlusion for 24 hours



Capsaicin 117

C


Early lesion (up to 6 weeks of age): blanch-ing of the involved skin; development offine telangiectasias; formation of a red orviolaceous macule or papule, often sur-rounded by a faint whitish haloProliferative stage (up to 12 months): dome-shaped, multilobular papule or nodule; maydevelop central erosion or ulceration; firm,rubbery consistency; expands withincreased intravascular pressureInvolution stage: shrinks centrifugally fromthe center of the lesion; lesion becomes lessred, with a dusky maroon to purple color;eventually regains normal flesh tones(“graying”)Cavernous variant: deep dermal and subcu-taneous red-to-violaceous nodule; regres-sion is often incomplete


Nevus flammeus; blue rubber bleb nevussyndrome; Mafucci syndrome; angiosar-coma arteriovenous malformation; infan-tile fibrosarcoma; infantile myofibromato-sis; pseudo-Kaposi’s hemangioendotheli-oma; lymphatic malformation; teratoma;Gorham syndrome; Riley-Smith syndrome

Therapy

Ulcerated hemangiomas and thin superfi-cial hemangiomas – flash lamp-pumpedpulsed dye laser; lesions compromisingfunction (e.g. larynx or eyelid) – pred-nisolone 2–5 mg per kg per day PO

References

Richards KA, Garden JM (2000) The pulsed dye laser for cutaneous vascular and nonvascular lesions. Seminars in Cutaneous Medicine & Surgery 19(4):276–286

Capsaicin

Trade name(s)Zostrix; Zostrix HP


Drug classAnalgesic

Mechanism of actionDepletes substance P and prevents re-accu-mulation in peripheral neurons

Dosage form0.025% cream, gel, lotion; roll-on; 0.075%cream, gel, lotion; roll-on


Common side effectsCutaneous: burning sensation; erythema


Capsaicin. Dermatologic indications and dosage


Brachioradial pruritus Apply 4–5 times daily for first 1–3 weeks; then taper as per therapeutic response

Apply 4–5 times daily for first 1–3 weeks; then taper as per therapeutic response

Notalgia paresthetica Apply 4–5 times daily for first 1–3 weeks; then taper as per therapeutic response


Post herpetic neuralgia




118 Carate



ReferencesSugeng MW, Yosipovitch G, Leok GC (2001) Post

herpetic neuralgia and the dermatologist. In-ternational Journal of Dermatology 40(1):6–11>

Carate

� Pinta

Carbon baby

� Universal acquired melanosis

Carbuncle

� Furuncle

Carcinoid syndrome

� Malignant carcinoid syndrome

Carcinoma cuniculatum

� Verrucous carcinoma

Carcinoma in situ


Carcinoma in situ of the penis

� Erythroplasia of Queyrat

Cardiocutaneous lentiginosis syndrome

� LEOPARD syndrome

Cardiocutaneous syndrome


Carducci fever

� Boutonneuse fever

Carney myxoma-endocrine complex

� Carney’s syndrome


Carrión’s disease 119

C

Carney’s syndrome

Synonym(s)Carney myxoma-endocrine complex;myxoma-spotty pigmentation-endocrineoveractivity

DefinitionFamilial multiple neoplasia and lentigino-sis syndrome with primary pigmented nod-ular adrenocortical disease (PPNAD); pri-mary adrenal form of hypercortisolism,lentigines, ephelides, and blue nevi of theskin and mucosae and a variety of endo-crine and other types of tumors

PathogenesisAutosomal dominant trait; two geneticallydistinct forms: one type mapped to chro-mosome 17 (CNC type 1); second typemapped to chromosome 2

Clinical manifestationLentigines; nevocellular nevi; freckling;hamartomas of the oral cavity; striae;kyphosis; easy bruising; sparse or absenthair; atrial and skin myxomasEndocrinopathies: Cushing syndrome;acromegaly; hyperparathyroidism; prolac-tin-secreting tumor; multiple thyroid nod-ules

Differential diagnosisNevi; lentigines; McCune-Albright syn-drome; neurofibromatosis; ephelides

TherapyTreatment of the endocrine overactivity;surgical excision of symptomatic myxomas

ReferencesKiryu T, Kawaguchi S, Matsui E, Hoshi H, Kokubo

M, Shimokawa K (1999) Multiple chondroma-tous hamartomas of the lung: A case report and review of the literature with special reference to Carney syndrome. Cancer 85(12):2557–2261

Carotenemia

Synonym(s)None

DefinitionIncreased carotenoid pigments from inges-tion of foodstuffs containing these nutrients

PathogenesisDeposition of yellow-orange pigments inthe stratum corneum after the prolonged orexcessive consumption of carotene-richfoods

Clinical manifestationAsymptomatic, yellow-orange discolora-tion, particularly at sites with a thick stra-tum corneum, such as the palms and soles;no change in scleral pigmentation

Differential diagnosisJaundice; lycopenemia (orange-yellow skindiscoloration due to the ingestion of largeamounts of tomatoes); riboflavinemia; Add-ison’s disease; drug reaction (e.g. quinic-rine)

TherapyDecreased ingestion of carotene-rich foods

ReferencesLeung AK (1987) Carotenemia. Advances in Pedi-

atrics 34:223–248

Carrión disease

� Bartonellosis

Carrión’s disease

� Bartonellosis


120 Cat-scratch disease

Cat-scratch disease

� Bartonellosis

Cavernous hemangioma

� Capillary hemangioma

Cavernous lymphangioma

� Lymphangioma

CD30+ cutaneous large T-cell lymphoma

� Cutaneous CD30+ (Ki-1) anaplastic large-cell lymphoma

Cellulite

DefinitionFat deposits under the skin outwardly giv-ing the skin a dimpled or orange-peel-likeappearance

ReferencesDraelos ZD, Marenus KD (1997) Cellulite. Etiolo-

gy and purported treatment. Dermatologic Surgery 23(12):1177–1181

Cellulitis

Synonym(s)None

DefinitionPurulent inflammation of the deep dermisand subcutaneous tissue, most often sec-ondary to a bacterial infection

PathogenesisImmune reaction to invading bacteria withan inflammatory response in the dermisand subcutaneous tissues, resulting in signsof inflammation

Clinical manifestationFour signs of infection: erythema, pain,swelling, and warmth; imprecise margins ofinfection; areas of edema and erythemablending into the surrounding normal skin;systemic symptoms (e.g. fever, malaise);signs of lymphangitis with red lines extend-ing proximal from the area of inflamma-tion; regional lymphadenopathy; crepituswith anaerobic organisms

Differential diagnosisPanniculitis; stasis dermatitis; contact der-matitis; arthropod envenomation; burns;septic joints; erysipelas; ecthyma; gas gan-grene

TherapyOral antibiotic: dicloxacillin; cephalexin;azithromycin; clarithromycinSystemic antibiotic: nafcillin: adults – 0.5–1.5 gm IV every 4 hours for 3–7 days; chil-dren – 10–20 mg per kg IV every 4 hours for3–7 daysCefotaxime: adults – 1 gm IV every 12 hoursfor 3–7 days; children – 12.5–45 mg per kgIV every 6 hours for 3–7 days

ReferencesDanik SB, Schwartz RA, Oleske JM (1999) Celluli-

tis. Cutis 64(3):157–160,163–164

Central papillary atrophy

� Median rhomboid glossitis


Cephalothoracic dystrophy 121

C

Centrofacial lentiginosis


Cephalexin

Trade name(s)Keflex; Keftab; Biocef


Drug classCephalosporin antibiotic

Mechanism of actionInhibition of penicillin-binding proteins,which results in defective bacterial cell wallsynthesis

Dosage form250 mg, 500 mg tablet; 125 mg per 5 ml, 250mg per 5 ml suspension


Common side effectsCutaneous: skin eruption

Gastrointestinal: nausea, vomiting,diarrheaLaboratory: eosinophilia, elevated liverenzymesNeurologic: headache, dizziness

Serious side effectsBone marrow: thrombocytopenia, neutro-peniaGastrointestinal: pseudomembranous coli-tisImmunologic: anaphylaxis

Drug interactionsAminoglycoside antibiotics; oral contracep-tives; probenecid

Contraindications/precautionsHypersensitivity to drug class or compo-nent; caution if there is a history of penicil-lin allergy; caution with impaired renalfunction or if patient is lactating

ReferencesSadick N (2001) Systemic antibiotics. Dermato-

logic Clinics 19(1):1–24>

Cephalothoracic dystrophy


Cephalexin. Dermatologic indications and dosage


Cellulitis 250–500 mg PO 4 times daily for 7 days

25–100 mg per kg daily, divided into 4 doses for 7–10 days

Ecthyma 250–500 mg PO 4 times daily for 7 days


Erysipelas 250–500 mg PO 4 times daily for 7 days


Impetigo 250–500 mg PO 4 times daily for 7 days


Scarlet fever 250–500 mg PO 4 times daily for 7 days



122 Cercarial dermatitis

Cercarial dermatitis

Synonym(s)Swimmer’s itch; bather’s itch; clam digger’sitch; silt itch; swamp itch; sedge pool itch

DefinitionPruritic eruption from an inflammatoryreaction to schistosomal cercariae at thepoint of entry

PathogenesisSnail as primary host for schistosomal cer-cariae; free-swimming organisms penetrateinto human skin and fail to complete lifecycle; inflammatory reaction to the organ-ism causes inflammation

Clinical manifestationLocalized pruritus followed by red maculesand papules; occurring mainly in exposedparts of the skin; inflammatory responsepeaking at 2–3 days and subsiding in 1–2weeks

Differential diagnosisSeabather’s eruption; insect bite reaction;harvest mite infestation; creeping eruption

TherapyIce compresses applied for 15–20 minutes 2–4 times per day; mid potency topical corti-costeroids; antihistamines, first generation,for nighttime sedation

ReferencesFolster-Holst R, Disko R, Rowert J, Bockeler W,

Kreiselmaier I, Christophers E (2001) Cercarial dermatitis contracted via contact with an aquarium: case report and review. British Jour-nal of Dermatology 145(4):638–640

Cervical lipomatosis

� Benign symmetric lipomatosis

Chagas disease

� American trypanosomiasis

Chalazion

Synonym(s)Meibomian cyst

DefinitionGranuloma of either meibomian gland orZeis gland of the eyelid

PathogenesisLipid-breakdown products from retainedglandular secretions resulting in granula-tion tissue and inflammation; bacterialenzyme actions possibly part of the process

Clinical manifestationFirm, red papule of the lid; associated withseborrheic dermatitis, chronic blepharitis,and rosacea

Differential diagnosisHordeolum; sebaceous neoplasm; orbitalcellulitis; marginal cyst; mucocele; hydro-cystoma; oncocytoma

TherapyMedical therapy: moist heat applied twicedaily for 15–30 minutesSurgical therapy: incision and drainage offluctuant lesions; drainage via a transcon-junctival incision and curettage

ReferencesLederman C, Miller M (1999) Hordeola and

chalazia. Pediatrics in Review 20(8):283–284

Chanarin Dorfman disease

� Chanarin-Dorfman syndrome


Chancroid 123

C

Chanarin-Dorfman syndrome

Synonym(s)Chanarin Dorfman disease; Dorfman Cha-narin syndrome; ichthyosiform erythro-derma with vacuolation; ichthyotic neutrallipid storage disease; neutral lipid storagedisease; triglyceride storage disease

DefinitionHereditary disorder of lipid metabolism,characterized by ichthyosis, myopathy, andabnormal white blood cells with vacuolesfilled with lipids

PathogenesisPrecise defect unknown; autosomal reces-sive trait; inability to break down intracellu-lar triglycerides

Clinical manifestationModerate, generalized erythema and scale;myopathy; psychomotor delay; cataracts;decreased hearing

Differential diagnosisCongenital ichthyosiform erythroderma;Refsum’s disease

TherapyAlpha hydroxy acids

ReferencesWessalowski R, Schroten H, Neuen-Jacob E, Re-

ichmann H, Melnik BC, Lenard HG, Voit T (1994) Multisystem triglyceride storage disor-der without ichthyosis in two siblings. Acta Paediatrica 83(1):93–98

Chancre

DefinitionPainless ulcer characterizing primary syph-ilis

ReferencesGoens JL, Janniger CK, De Wolf K (1994) Derma-

tologic and systemic manifestations of syphilis. American Family Physician 50(5):1013–1020

Chancroid

Synonym(s)Soft chancre

DefinitionSexually transmitted genital disease, causedby the gram-negative bacillus Haemophilusducreyi, characterized by painful genitalulcers and inflammatory inguinal adenopa-thy

PathogenesisCaused by gram-negative bacillus Haemo-philus ducreyi; organism produces a potentdistending toxin, probably contributing tothe production and slow healing of ulcers

Clinical manifestationDisease in men: painful, erythematouspapules at the site of recent sexual contact;foreskin most common site of infection, butoccasionally occurring on the shaft, glans,or meatus of the penis; lesions become pus-tular and then ulcerate; associated withregional lymphadenopathy; constitutionalsymptoms, such as malaise and low-gradefeversDisease in women: ulcers most commonlyoccur on the labia majora but sometimesalso on the labia minora, thigh, perineum,or cervix; lesions usually less symptomaticthan in men

Differential diagnosisSyphilis; lymphogranuloma venereum; her-pes simplex virus infection; traumaticulceration; aphthae; Behçet’s disease;Crohn’s disease; fixed drug reaction

TherapyAzithromycin; ciprofloxacin; ceftriaxone250 mg IM for 1 dose


124 Charbon

ReferencesBrown TJ, Yen-Moore A, Tyring SK (1999) An

overview of sexually transmitted diseases. Part I. Journal of the American Academy of Derma-tology 41(4):511–532

Charbon


Cheadle-Möller-Barlow syndrome

� Barlow’s disease

Chédiak-Higashi syndrome

Synonym(s)Bequez Cesar syndrome, Chédiak-Stein-brinck-Higashi syndrome

DefinitionDisorder characterized by immune defi-ciency, partial oculocutaneous albinism,easy bruising, and bleeding, as a result ofdeficient platelets and recurrent infections

PathogenesisAutosomal recessive trait; gene mutationaffecting the synthesis and/or maintenanceof storage/secretory granules in varioustypes of cells, including melanocytes andneutrophils; abnormal intracellular proteintransport

Clinical manifestationLack of skin pigmentation, similar to albi-nos, but in patchy distribution; blonde hair;blue eyes; photophobia; gingivitis and oralmucosal ulceration; frequent and severepyogenic infections; neurologic dysfunction

Differential diagnosisOculocutaneous albinism; Griscelli syn-drome; postinflammatory hypopigmenta-tion; poliosis; piebaldism

TherapyBone marrow transplantation�

ReferencesStolz W, Graubner U, Gerstmeier J, Burg G, Belo-

hradsky BH (1989) Chediak-Higashi syndrome: Approaches in diagnosis and treatment. Current Problems in Dermatology 18:93–100

Chédiak-Steinbrinck-Higashi syndrome

� Chédiak-Higashi syndrome

Cheilitis

DefinitionDryness, chapping, and fissuring of the lip

ReferencesKaugars GE, Pillion T, Svirsky JA, Page DG, Burns

JC, Abbey LM (1999) Actinic cheilitis: A review of 152 cases. Oral Surgery Oral Medicine Oral Pathology Oral Radiology & Endodontics 88(2):181–186

Cheilitis, actinic


Cheilitis, angular

� Angular cheilitis


Cherry angioma 125

C

Cheilitis granulomatosa

Synonym(s)Miescher-Melkersson-Rosenthal syn-drome; granulomatous cheilitis; orofacialgranulomatosis; Miescher’s cheilitis granu-lomatosa

DefinitionChronic, non-tender swelling of the lip dueto granulomatous inflammation; Melkers-son-Rosenthal syndrome: chronic swellingof the lip, facial palsy, and lingua plicata

PathogenesisUnknown stimulus to granuloma forma-tion; swelling secondary to edema andgranulomas in the lamina propria

Clinical manifestationFirst episode of edema resolves completelyin hours or days; after recurrent attacks,occasional constitutional symptoms withattacks; swelling sometimes persists andbecomes permanent; recurrences commonfrom days to years; affected lip cracks andfissures, with reddish-brown discolorationand scaling; slow regression over severalyears

Differential diagnosisSarcoidosis; dental abscess; angioedema; liptrauma; insect bite reaction; Crohn’s disease

TherapyTriamcinolone 3–4 mg per ml intralesional;clofazimine 100 mg PO twice daily for 10days, then twice weekly for 4 months; met-ronidazole 500 mg PO twice daily

ReferencesRidder GJ, Fradis M, Lohle E (1997) Cheilitis gran-

ulomatosa Miescher: treatment with clofaz-imine and review of the literature. Annals of Otology, Rhinology & Laryngology 110(10):964–967

Cheiloid

� Keloid

Cheilosis

� Cheilitis

Chemotherapy-induced alopecia

� Anagen effluvium

Cherry angioma

� Cherry hemangioma

Cheilitis granulomatosa. Infiltrated lower vermillion portion of the lip, with secondary irritant dermatitis of the cutaneous portion of the lip


126 Cherry hemangioma

Cherry hemangioma

Synonym(s)Cherry angioma; Campbell de Morganspots; senile angioma

DefinitionBenign growth of the skin formed by a pro-liferation of dilated venules

PathogenesisUnknown

Clinical manifestationSmall, red-to-violaceous macule, or a largerdome-shaped or polypoid papule; occurson all body sites except mucous mem-branes; increases in number and size withadvancing age

Differential diagnosisAngiokeratoma; petechiae; thrombocytope-nia; Kaposi’s sarcoma; bacillary angiomato-sis; vasculitis; benign pigmented purpura;insect bite reaction; blue rubber bleb nevussyndrome

TherapyDestruction by electrodesiccation andcurettage; liquid nitrogen cryotherapy;pulse dye laser ablation; CO2 laser vapori-zation

ReferencesSala F, Crosti C, Menni S, Piccinno R (1984) Cher-

ry hemangioma: An SEM study. Journal of Cu-taneous Pathology 11(6):531–533

Cheveux incoiffables

� Uncombable hair syndrome

Chickenpox

� Varicella

Chiclero’s ulcer

DefinitionType of leishmaniasis of the skin, primarilyaffecting men who visit the forests to col-lect chicle (gum); forms an ulcerating lesionon the ear lobe

ReferencesAndrade-Narvaez FJ, Simmonds-Diaz E, Rico-

Aguilar S, Andrade-Narveez M, Palomo-Cetina A, Canto-Lara SB, Garcia-Miss MR, Madera-Sevilla M, Albertos-Alpuche N (1990) Inci-dence of localized cutaneous leishmaniasis (chiclero's ulcer) in Mexico. Transactions of the Royal Society of Tropical Medicine & Hygiene 84(2):219–220

Chilblains

Synonym(s)Pernio; perniosis

DefinitionInflammatory skin condition presenting aspruritic and/or painful acral lesions afterexposure to cold

PathogenesisAbnormal vascular response to cold expo-sure

Clinical manifestationRecurrent, painful and/or pruritic, red-to-violaceous papules or nodules on the fin-


Chloracne 127

C

gers and/or toes; sometimes vesiculating orulcerating; occurs 12–24 hours after coldexposure; sometimes occurs in associationwith systemic diseases, including chronicmyelomonocytic leukemia, anorexia ner-vosa, dysproteinemias, macroglobuline-mia, cryoglobulinemia, cryofibrinogene-mia, cold agglutinins, antiphospholipidantibody syndrome, or Raynaud disease

Differential diagnosisVasculitis; sarcoidosis; erythema multi-forme; acrocyanosis; septic or cholesterolemboli; erythromelalgia; polycythemiavera; purple toe syndrome secondary tocoumarin; Raynaud phenomenon

TherapyProphylactic warming of acral areas withminimization of cold exposure; UVB photo-therapy

ReferencesCarruthers R (1988) Chilblains (perniosis). Aus-

tralian Family Physician 17(11):968–969

CHILD syndrome

Synonym(s)Congenital hemidysplasia; ichthyosiformnevus

DefinitionVariant of ichthyosiform erythrodermacharacterized by congenital hemidysplasia,unilateral ichthyosiform erythroderma, andlimb defects

PathogenesisSuggestion that peroxisomal deficiency ininvolved skin leads to accumulation ofPGE2, resulting in keratinocyte growth andepidermal hyperproliferation; mosaicismpossibly accounts for unilateral distribution

Clinical manifestationUnilateral, scaly, erythematous plaqueswith a sharp midline demarcation, usuallypresent at birth or early infancy; nail dys-trophy; ipsilateral limb defects; ipsilateralhypoplasia of the brain, lung, thyroid andreproductive tract

Differential diagnosisCongenital ichthyosiform erythroderma;inflammatory linear verrucous epidermalnevus (ILVEN); epidermal (organoid)nevus syndrome (Schimmelpfennig-Feuer-stein-Mims); phacomatosis pigmentokera-totica


ReferencesHapple R, Mittag H, Kuster W (1995) The CHILD

nevus: A distinct skin disorder. Dermatology 191(3):210–216

Chloasma

� Melasma

Chloracne

Synonym(s)Occupational acne

DefinitionAcneform eruption, with a preponderanceof comedones, after exposure to chlorin-ated hydrocarbons, found in herbicidemanufacturing and cable splicing, andpolychlorinated biphenyls

PathogenesisUnknown


128 Chlorpheniramine

Clinical manifestationSmall, flesh-colored cysts and comedones,associated with pruritus, involving the face,postauricular region, and angles of the jaw;but sparing the nose and malar regions

Differential diagnosisAcne vulgaris; syndrome of Favre-Racou-chot; acne cosmetica; steroid-induced acne;pomade acne; tropical acne; radiation acne;gram negative folliculitis

TherapyIsotretinoin�; tretinoin; tetracycline; inci-sion and drainage; avoidance of agents con-taining chlorinated hydrocarbons

ReferencesRosas Vazquez E, Campos Macias P, Ochoa Tirado

JG, Garcia Solana C, Casanova A, Palomino Moncada JF (1996) Chloracne in the 1990s. In-ternational Journal of Dermatology 35(9):643–645

Chlorpheniramine


Chondrodermatitis nodularis chronica antihelicis

� Chondrodermatitis nodularis helicis

Chondrodermatitis nodularis chronica helicis

� Chondrodermatitis nodularis helicis

Chondrodermatitis nodularis helicis

Synonym(s)Chondrodermatitis nodularis chronica heli-cis; chondrodermatitis nodularis chronicaantihelicis

DefinitionInflammatory condition of the ear produc-ing painful papules and nodules

PathogenesisPossibly involves dermal inflammationfrom trauma, cold, actinic damage, or pres-sure

Clinical manifestationFirm, tender, well demarcated papule, witha raised, rolled edge and central erosion orulceration; develops on the most promi-nent projection of the ear, most commonlyon the apex of the helix; distribution on theantihelix more common in women

Differential diagnosisActinic keratosis; basal cell carcinoma;squamous cell carcinoma; keratoacan-thoma; tophus; rheumatoid nodule; colloidmilium; endochondral pseudocyst

Chondrodermatitis nodularis helicis. Flesh-colored papule with punctate central erosion on the underside of the pinna of the ear


Chromoblastomycosis 129

C

TherapyCryotherapy; triamcinolone 3–5 mg per mlintralesional; surgical excision; CNH pillowto relieve pressure

ReferencesBeck MH (1985) Treatment of chondrodermatitis

nodularis helicis and conventional wisdom? British Journal of Dermatology 113(4):504–505

Chondrodysplasia punctata

� Conradi disease

Chondrodystrophia calcificans congenita

� Conradi disease

Choristoma

� Dermoid cyst

Christ-Siemens-Touraine syndrome

� Anhidrotic ectodermal dysplasia

Chromhidrosis

Synonym(s)Ephidrosis tincta; eccrine chromhidrosis

DefinitionCondition characterized by colored sweat,mostly secondary to colored apocrinesecretions

PathogenesisElevated levels of lipofuscins possiblyinvolved; substance P possibly an impor-tant neurotransmitter; extrinsic contribut-ing factors include dyes, chromogenic bac-teria, and chemical contactants

Clinical manifestationTurbid, yellow, red, blue, or green apocrinesecretion; color accentuated in the pores

Differential diagnosisHyperbilirubinemia; pseudomonas infec-tion; poisoning; alkaptonuria; bleeding dia-thesis (red sweat, hematohidrosis); copperexposure (blue sweat)

TherapyCapsaicin 0.025% cream applied 4–5 timesper day

ReferencesMarks JG Jr (1989) Treatment of apocrine chrom-

hidrosis with topical capsaicin. Journal of the American Academy of Dermatology 21(2 Pt 2):418–420

Chromoblastomycosis

Synonym(s)Chromomycosis; verrucous dermatitis;phaeohyphomycosis; cystic chromomycosis

DefinitionChronic skin and subcutaneous fungalinfection caused by one of multiple fungalpathogens

PathogenesisInoculation by one of the following: Hor-modendrum pedrosoi, H. compactum, or


130 Chromomycosis

Phialophora verrucosa; organisms isolatedfrom wood and soil

Clinical manifestationAsymptomatic, verrucous papule, slowlyenlarging to large plaque or thick nodule;lesions often ulcerate; satellite lesions pro-duced by autoinoculation

Differential diagnosisNorth American blastomycosis; SouthAmerican blastomycosis; tuberculosis;leishmaniasis; syphilis; yaws; squamous cellcarcinoma; atypical mycobacterial infec-tion; sporotrichosis; nocardiosis

TherapyItraconazole; terbinafine; flucytosine withor without localized hyperthermia; cryo-therapy; surgical excision for small lesions

ReferencesRivitti EA, Aoki V (1999) Deep fungal infections

in tropical countries. Clinics in Dermatology 17(2):171–190

Chromomycosis

� Chromoblastomycosis

Chromophytosis

� Tinea versicolor


Synonym(s)Actinic reticuloid; persistent light reactiv-ity; photosensitive eczema; photosensitivitydermatitis; persistent light reaction

DefinitionPersistent eczematous eruption in the sun-exposed areas of greater than 3 months'duration, with abnormal sensitivity toeither ultraviolet or visible light

PathogenesisDelayed type hypersensitivity reactioninvolving a light-induced immune response

Clinical manifestationEczematous and infiltrated plaques thatinvolve mostly exposed skin, but may gen-eralize to erythroderma

Differential diagnosisPolymorphous light eruption; allergic con-tact dermatitis; photocontact dermatitis;solar urticaria; actinic prurigo; atopic der-matitis; lupus erythematosus; cutaneous Tcell lymphoma

TherapyProtection from sunlight; photochemother-apy; azathioprine; hydroxychloroquine sul-fate; cyclosporine

ReferencesLim HW, Morison WL, Kamide R, Buchness MR,

Harris R, Soter NA (1994) Chronic actinic der-matitis. An analysis of 51 patients evaluated in the United States and Japan. Archives of Der-matology 130(10):1284–1289

Chronic adrenal insufficiency


Chronic atrophic acrodermatitis

� Acrodermatitis chronica atrophicans


Chronic granulomatous disease of childhood 131

C

Chronic atrophic polychondritis

� Relapsing polychondritis

Chronic bullous dermatosis of childhood

� Linear IgA dermatosis

Chronic bullous disease of childhood


Chronic cutaneous lupus erythematosus

� Lupus erythematosus, discoid

Chronic erythema nodosum

� Subacute nodular migratory pan-niculitis

Chronic granulomatous disease

Synonym(s)Chronic granulomatous disease of child-hood; fatal granulomatosis of childhood;

progressive septic granulomatosis; X-linkedchronic granulomatous disease

DefinitionInherited disorder of phagocytic cells, lead-ing to recurrent, life-threatening bacterialand fungal infections

PathogenesisFailure of phagocytes to generate sufficientquantities of reactive oxygen species;molecular defect represents a mutation inthe gene encoding the b subunit of cyto-chrome b558 (CYBB), located on the X chro-mosome

Clinical manifestationEarly onset of severe recurrent bacterialand fungal infections, often involving theskin; lungs most common site of infection;other involved sites include gastrointesti-nal tract, lymph nodes, liver, and spleen

Differential diagnosisBruton agammaglobulinemia; common var-iable immunodeficiency; severe combinedimmunodeficiency; HIV infection; comple-ment deficiency; leukocyte adhesion defi-ciency; Wiskott-Aldrich syndrome

TherapyProphylaxis of bacterial infections with tri-methoprim-sulfamethoxazole 5 mg per kgper day PO divided into 2 doses; bone mar-row transplantation�

ReferencesGoldblatt D, Thrasher AJ, Chronic granulomatous

disease. Clinical & Experimental Immunology 122(1):1–9

Chronic granulomatous disease of childhood

� Chronic granulomatous disease


132 Chronic hair pulling

Chronic hair pulling

� Trichotillomania

Chronic papulopustular facial dermatitis

� Perioral dermatitis

Chronic superficial dermatitis


Chrysiasis

Synonym(s)Chrysoderma

DefinitionDevelopment of a blue-gray pigmentationin skin and mucous membranes, caused byexposure to gold compounds

PathogenesisDeposition of gold salts in the dermis;increased melanin production in the epi-dermis

Clinical manifestationBlue-gray or violaceous hue to sun-exposedskin and sclerae; mucous membranesspared; pigmentation usually permanent;occurs only after a cumulative dose of atleast 50 mg per kg

Differential diagnosisArgyria; other drug-induced pigmentation(e.g. minocycline; amiodarone); Addison’s

disease; hemosiderosis; jaundice; carotene-mia; hemochromatosis


ReferencesSmith RW, Cawley MI (1997) Chrysiasis. British

Journal of Rheumatology 36(1):3–5

Chrysoderma

� Chrysiasis

Churg-Strauss disease


Churg-Strauss granulomatosis syndrome


Churg-Strauss syndrome

Synonym(s)Allergic granulomatosis; allergic angiitisand granulomatosis; eosinophilic granulo-matous vasculitis; Churg-Strauss granulo-matosis syndrome; granulomatous vasculi-tis with asthma

DefinitionDisorder characterized by asthma, tran-sient pulmonary infiltrates, eosinophilia,and systemic vasculitis

PathogenesisActivated eosinophils possibly pathogenic


Ciclopirox 133

C

Clinical manifestationCutaneous findings: red papules and mac-ules; palpable purpuric papules andplaques; cutaneous and subcutaneouspapules and nodulesRespiratory tract findings: allergic rhinitis;asthma; transient pulmonary infiltratesVasculitis target organs: kidney, heart, cen-tral nervous system, gastrointestinal tract

Differential diagnosisHenoch-Schönlein purpura; lupus ery-thematosus; bronchopulmonary aspergillo-sis; lymphoma; Loeffler syndrome; lympho-matoid granulomatosis; polyarteritisnodosa; rheumatoid arthritis

TherapyPrednisone�; steroid-sparing agents: meth-otrexate; azathioprine 100–150 mg PO perday; cyclosporine; cyclophosamide pulsetherapy 0.6 gm per m2 IV monthly for up to1 year

ReferencesGross WL (2002) Churg-Strauss syndrome: up-

date on recent developments. Current Opinion in Rheumatology 14(1):11–14


Synonym(s)Benign mucous membrane pemphigoid;scarring pemphigoid; mucosal pemphigoid

DefinitionAutoimmune vesiculobullous disease pre-dominately affecting mucous membranes

PathogenesisIgG antibodies against antigens in base-ment zone; major antigens associated areBPAG2 and epiligrin (laminin 5); immunereaction causes loss of adhesion at the der-mal-epidermal junction and blisters

Clinical manifestationPersistent, painful erosions on mucousmembranes, often healing with scarring;ocular involvement: pain or the sensation ofgrittiness in the eye; conjunctival inflam-mation and erosions; keratinization of theconjunctiva and shortening of the fornices;entropion with subsequent trichiasis; skin:tense vesicles or bullae, sometimes hemor-rhagic, sometimes healing with scarring ormilia; scalp involvement leads to alopecia

Differential diagnosisBullous pemphigoid; linear IgA dermatosis;erythema multiforme; Stevens-Johnsonsyndrome; epidermolysis bullosa; epider-molysis bullosa acquisita; dermatitis herpe-tiformis; impetigo; pemphigus foliaceus;pemphigus vulgaris; herpes simplex virusinfection; herpes zoster

TherapyLimited disease: mid potency topical corti-costeroid gel for mucous membranesExtensive disease: prednisone, dapsone;cyclophosphamide; azathioprine

ReferencesFleming TE, Korman NJ (2000) Cicatricial pem-

phigoid. Journal of the American Academy of Dermatology 43(4):571–591

Ciclopirox

Trade name(s)Loprox; Penlac

Generic availableNo

Drug classTopical antifungal agent

Mechanism of actionAffects synthesis of fungal cell wall


134 Ciprofloxacin

Dosage form0.77% cream, gel, lotion; 8% nail lacquer


Common side effectsCutaneous: burning, itching, redness, swell-ing




ReferencesGupta AK, Baran R (2000) Ciclopirox nail lacquer

solution 8% in the 21st century. Journal of the American Academy of Dermatology 43(4 Sup-plement):S96–102

Ciprofloxacin

Trade name(s)Cipro

Generic availableNo

Drug classFluoroquinolone antibiotic

Mechanism of actionInhibition of bacterial DNA gyrase, whichresults in interference with DNA replication

Dosage form100 mg, 250 mg, 500 mg, 750 mg tablet; 250,500 mg per 5 ml for intravenous infusion


Common side effectsCutaneous: photosensitivity, urticaria, orother vascular reactionGastrointestinal: nausea and vomiting,diarrhea, abdominal painNeurologic: agitation, confusion, insomnia,headache, dizziness, restlessness

Serious side effectsGastrointestinal: pseudomembranous coli-tisNeurologic: toxic psychosis, seizures

Drug interactionsAntacids; caffeine; calcium salts; clozapine;oral contraceptives; cyclosporine; glybu-ride/metformin; iron salts; non-steroidalanti-inflammatory drugs; olanzapine;phenytoin; probenecid; theophylline; warfa-rin

Ciclopirox. Dermatologic indications and dosage


Onychomycosis Apply Penlac once daily for up to 48 weeks

Apply Penlac once daily for up to 48 weeks

Tinea corporis Apply Loprox twice daily Apply Loprox twice daily

Tinea cruris Apply Loprox twice daily Apply Loprox twice daily

Tinea faciei Apply Loprox twice daily Apply Loprox twice daily

Tinea nigra Apply Loprox twice daily Apply Loprox twice daily

Tinea pedis Apply Loprox twice daily Apply Loprox twice daily

White piedra Apply Loprox twice daily Apply Loprox twice daily


Clarithromycin 135

C

Contraindications/precautionsHypersensitivity to drug class or compo-nent; safety not established for patients < 18years old; caution in those with impairedrenal or liver function; caution in thosewith seizures



Circumscribed neurodermatitis

� Lichen simplex chronicus

Circumscribed scleroderma

� Morphea

Clam digger’s itch


Clarithromycin

Trade name(s)Biaxin

Generic availableNo


Mechanism of actionInhibits protein synthesis of sensitive bacte-rial organisms

Dosage form250 mg, 500 mg tablet


Common side effectsCutaneous: skin eruption, vaginitisGastrointestinal: nausea, vomiting, abdom-inal pain, diarrhea, anorexia

Serious side effectsCutaneous: anaphylaxis, Stevens-Johnsonsyndrome, toxic epidermal necrolysisGastrointestinal: pseudomembranous coli-tis, cholestatic jaundice

Ciprofloxacin. Dermatologic indications and dosage


Cellulitis 250–500 mg PO twice daily for 7–21 days, depending on response

Not indicated

Chancroid 500 mg PO twice daily for 3 days Not indicated

Malakoplakia 250–500 mg PO for 7–14 days Not indicated

Mycobacterium marinum infection

500 mg 1–2 times daily for 4–6 weeks

Not indicated

Rhinoscleroma 250–500 mg PO twice daily for months to years

Not indicated

Rickettsialpox 250–500 mg PO daily for 5 days Not indicated

Salmonellosis 500 mg IV twice daily, then switch to PO when tolerated for a total course of 10–14 days

Not indicated


136 Clark’s nevus

Drug interactionsAmiodarone; antacids; budesonide; bus-pirone; carbamazepine; clozapine; oral con-traceptives; cyclosporine; digoxin; ergotalkaloids; methadone; phenytoin; pimoz-ide; protease inhibitors; quinidine; statins;tacrolimus; theophylline; valproic acid;vinca alkaloids; warfarin

Contraindications/precautionsHypersensitivity to drug class or compo-nent; caution in those with impaired liverfunction; do not use concomitantly withterfenadine or astemizole



Clark’s nevus

� Atypical mole

Classic typhus

� Epidemic typhus

Clavus

Synonym(s)Callus; callosity; corn, heloma, callous

DefinitionThickening of the skin due to intermittentpressure and frictional forces

PathogenesisInappropriate distribution of pressure ontoa specific site, producing increased fric-tional forces and reactive skin thickening

Clinical manifestationThickened skin, with retained skin derma-toglyphics, most commonly on the foot;occasional secondary maceration and fun-gal or bacterial infection

Clarithromycin. Dermatologic indications and dosage


Atypical mycobacterial infection

500 mg PO twice daily for 6–12 weeks after clinical remission

> 45 kg weight; 7.5 mg per kg PO twice daily for 6–12 weeks after clinical remission

Bacillary angiomatosis 250 mg PO twice daily for 3 weeks > 45 kg weight; 7.5 mg per kg PO twice daily for 3 weeks

Bartonellosis 250 mg PO twice daily for 3 weeks > 45 kg weight; 7.5 mg per kg PO twice daily for 3 weeks

Cellulitis 250 mg PO twice daily for 5–7 days > 45 kg weight; 7.5 mg per kg PO twice daily for 5–7 days

Ecthyma 250 mg PO twice daily for 5–7 days > 45 kg weight; 7.5 mg per kg PO twice daily for 5–7 days

Erythrasma 1 gm PO for 1 dose > 45 kg weight; 7.5 mg per kg PO for 1 dose

Impetigo 250 mg PO twice daily for 5–7 days > 45 kg weight; 7.5 mg per kg PO twice daily for 5–7 days


500 mg PO twice daily for 6–12 weeks

15 mg per kg PO divided into 2 doses daily for 6–12 weeks


Clindamycin, systemic 137

C

Differential diagnosisWart; gout; lichen planus; interdigital neu-roma; lichen simplex chronicus; palmo-plantar keratoderma; keratosis punctata;porokeratosis plantaris

TherapyMechanical pressure redistribution: orthot-ics; well-fitted shoes; protective pads onpressure points; skin-surface paring forsymptomatic lesions

ReferencesFreeman DB (2002) Corns and calluses resulting

from mechanical hyperkeratosis. American Family Physician 65(11):2277–2280


Synonym(s)Clear cell acanthoma of Degos; Degos’acanthoma; acanthome à cellules claires

DefinitionSkin tumor with accumulation of clear, gly-cogen-containing cells

PathogenesisUnknown

Clinical manifestationSolitary, dome-shaped papule or nodule,with a peripheral scale; occurring mostcommonly on the lower extremities

Differential diagnosisHistiocytoma; seborrheic keratosis;lichenoid keratosis; pyogenic granuloma;amelanotic melanoma


ReferencesDegos R, Civatte J (1970) Clear-cell acanthoma.

Experience of 8 years. British Journal of Der-matology 83(2):248–254

Clear cell acanthoma of Degos

� Clear cell acanthoma

Clear cell adenoma


Clear cell hidradenoma


Clear cell myoepithelioma

� Eccrine hidradenoma

Climatic bubo

� Lymphogranuloma venereum

Clindamycin, systemic

Trade name(s)Cleocin


Drug classLincosamide antibiotic

Mechanism of actionBinds to bacterial 50S ribosomal subunit,interfering with protein synthesis


138 Clostridial myonecrosis

Dosage form75 mg, 150 mg tablet; intramuscular prepa-ration; solution for intravenous injection


Common side effectsCutaneous: skin eruption, pruritusGastrointestinal: nausea, vomiting,diarrhea, abdominal pain, jaundice

Serious side effects,Bone marrow: thrombocytopenia; granulo-cytopeniaCutaneous: anaphylaxis, Stevens-JohnsonsyndromeGastrointestinal: pseudomembranous coli-tis, esophagitis

Drug interactionsOral contraceptives; neuromuscular block-ers

Contraindications/precautionsHypersensitivity to drug class or compo-nent; history of ulcerative colitis; cautionwith renal or hepatic impairment

ReferencesWeingarten-Arams J, Adam HM (2002) Clin-

damycin. Pediatrics in Review 23(4):149–150

Clostridial myonecrosis

� Gas gangrene

Clouston’s disease

� Hidrotic ectodermal dysplasia

Clubbing of the nails

DefinitionA broadening and thickening of the fingersor toes, with increased lengthwise curva-ture and curvature of the tip of the nail, andflattening of the angle between the cuticleand nail

ReferencesCollins KP, Burkhart CG (1985) Clubbing of the

fingers. International Journal of Dermatology 24(5):296–297

Cobb syndrome

Synonym(s)Cutaneomeningospinal angiomatosis

Clindamycin, systemic. Dermatologic indications and dosage


Acne vulgaris 150 mg PO 2–3 times daily Not indicated

Gas gangrene 15 mg per kg IV daily divided into 3 doses

10 mg per kg daily IV divided into 3 doses

Necrotizing fasciitis 600–900 mg IV every 6–12 hours 25–40 mg per kg IV divided into 3–4 doses daily

Paronychia, acute 150 mg PO 3 times daily for 7–10 days

Not indicated

Streptococcal toxic shock-like syndrome

600–900 mg IV every 6–12 hours 25–40 mg per kg IV divided into 3–4 doses daily


Coccidiosis 139

C

DefinitionAssociation of spinal angiomas or arteriov-enous malformations with congenital cuta-neous vascular lesions in the same der-matome

PathogenesisApparently a developmental abnormality ofthe vessels of the spinal cord and skin

Clinical manifestationVascular abnormalities, including asympto-matic port wine stain, angiokeratoma, orhemangioma; various neurologic findingsdepending on level of the vascular abnor-mality; associated scoliosis or kyphoscolio-sis

Differential diagnosisNevus flammeus; infantile hemangioma;Sturge-Weber syndrome; Wyburn-Masonsyndrome; Klippel-Trenaunay-Weber syn-drome; angiokeratoma corporis diffusum

TherapyNeurosurgical evaluation

ReferencesShim JH, Lee DW, Cho BK (1996) A case of Cobb

syndrome associated with lymphangioma cir-cumscriptum. Dermatology 193(1):45–47

Coccidioidomycosis

Synonym(s)Valley fever, San Joaquin Valley fever;desert rheumatism; coccidiosis

DefinitionDisease caused by the spores of the fungus,Coccidioides immitis

PathogenesisInhalation of arthroconidia from the organ-ism C. immitis; sometimes spreadingwithin the lungs or via the bloodstream;rare direct skin inoculation of C immitis

Clinical manifestationProdrome of fever, weight loss, malaise, andheadache; acute or subacute pneumonic ill-ness most common clinical presentation,with cough and inspiratory chest painNon-specific skin findings: erythema nodo-sum; erythema multiformeSpecific skin findings: superficial papules;keratotic nodules; verrucous ulcers; subcu-taneous fluctuant abscessesOther organs of dissemination: bones andjoints; adrenal glands; central nervous sys-tem; liver

Differential diagnosisRosacea; tuberculosis; sarcoidosis; actino-mycosis; leishmaniasis; Wegener’s granulo-matosis; vasculitis; syphilis; tinea faciei;sporotrichosis; chromoblastomycosis; par-apsoriasis; mycosis fungoides; lichen planus

TherapyDisseminated disease: Amphotericin B 0.3–1 mg per kg per day IV; start with 0.25 mgper kg per day and increased by 5–10 mgper day; fluconazole; itraconazole

ReferencesGalgiani JN (1997) Coccidioidomycosis. Current

Clinical Topics in Infectious Diseases 17:188–204

Coccidiosis

� Coccidioidomycosis

Coccidioidomycosis. Erythematous, edematous plaque on the upper nasal bridge


140 Cochin China diarrhea

Cochin China diarrhea

� Strongyloidosis

Cockade purpura with edema


Cockayne syndrome

Synonym(s)Cockayne’s syndrome; dwarfism with reti-nal atrophy and deafness

DefinitionDisorder characterized by sunlight sensitiv-ity, short stature, neurologic abnormalities,cataracts, and the appearance of prematureaging

PathogenesisDefective DNA repair, specifically tran-scription-coupled repair; two defectivegenes, CSA and CSB, coding for proteinsthat interact with components of the tran-scriptional machinery and with DNA repairproteins

Clinical manifestationGrowth failure; aged appearance; extremephotosensitivity; dental abnormalities; pro-gressive neurologic abnormalities, includ-ing mental retardation and deafness; degen-erative retinal pigmentary abnormalities

Differential diagnosisXeroderma pigmentosum, particularly theDeSanctis-Cacchione variant; Bloom’s syn-drome; progeria; Werner’s syndrome; Roth-mund-Thompson syndrome; ataxia-tel-angiectasia

TherapyStrict sunlight avoidance

ReferencesNance MA, Berry SA (1992) Cockayne syndrome:

review of 140 cases. American Journal of Medi-cal Genetics 42:68–84

Cockayne’s syndrome

� Cockayne syndrome

Colchicine

Trade name(s)None


Drug classAnti-inflammatory

Mechanism of actionBinds to dimers of tubulin, preventingmicrotubule assembly

Dosage form0.5 mg, 0.6 mg tablet


Common side effectsCutaneous: skin eruption, alopeciaGastrointestinal: diarrhea, nausea, vomit-ing, abdominal painHematologic: anemia, thrombophlebitis

Serious side effectsCutaneous: cellulitisHematologic: agranulocytosis, aplastic ane-mia, neutropeniaNeurologic: myoneuropathy


Cold panniculitis 141

C

Drug interactionsCyclosporine

Contraindications/precautionsHypersensitivity to drug class or compo-nent; blood dyscrasias; pregnancy; cautionin serious gastrointestinal disorders

ReferencesRitter S, George R, Serwatka LM, Elston DM

(2002) Long-term suppression of chronic Sweet's syndrome with colchicine. Journal of the American Academy of Dermatology 47(2):323–324

Cold panniculitis

Synonym(s)Popsicle panniculitis; Haxthausen’s disease

DefinitionAcute, nodular, erythematous eruption atskin sites exposed to the cold

PathogenesisLocalized cold injury leading to inflamma-tion of the subcutaneous adipose tissue;

infants possibly at greater risk because ofincreased fatty acid content in adipose tis-sue

Clinical manifestationBeginning 1–3 days after a cold injury toexposed or poorly protected areas; painful,firm, red or cyanotic, indurated noduleswith ill-defined margins; in obese patients,buttocks, thighs, arms, and area under thechin are most commonly affected; in smallchildren, is the site of involvement oftencheeks

Differential diagnosisSubcutaneous fat necrosis of the newborn;sclerema neonatorum; poststeroid pannicu-litis; erythema infectiosum; atopic dermati-tis; cellulitis

TherapyNone

ReferencesTer Poorten JC, Hebert AA, Ilkiw R (1995) Cold

panniculitis in a neonate. Journal of the Amer-ican Academy of Dermatology 33(2 Pt 2):383–385

Colchicine. Dermatologic indications and dosage


Acute neutrophilic dermatosis

0.6–1.8 mg PO daily Not established

Aphthous stomatitis 0.6–1.8 mg PO daily Not established

Behçet’s disease 0.6–1.8 mg PO daily Not established

Calcinosis cutis 0.6–1.8 mg PO daily Not established

Dermatomyositis 0.6–1.8 mg PO daily Not established



Linear IgA bullous dermatosis


Pachydermoperiostosis




Urticarial vasculitis 0.6–1.8 mg PO daily Not established


142 Cold urticaria

Cold urticaria

Synonym(s)None

DefinitionPhysical urticaria characterized by ery-thematous papules and plaques arisingwhen the body temperature cools

PathogenesisFamilial type: autosomal dominant trait;unknown causeAcquired type: unknown cause

Clinical manifestationPruritus, erythema, and urticaria precipi-tated by exposure to cold objects, cold air,or cold water; sometimes associated withconstitutional signs and symptoms, such asfever, chills, headache, myalgia, loss of con-sciousness; symptoms often disappear in afew months in the acquired type

Differential diagnosisAquagenic urticaria; dermatographism;anaphylaxis from foods, medications, etc.;cholinergic urticaria

TherapyAntihistamines, first generation, especiallycyproheptadine

ReferencesClaudy A (2001) Cold urticaria. Journal of Investi-

gative Dermatology Symposium Proceedings 6(2):141–142

Collagenoma

� Connective tissue nevus

Collagenoma perforant verruciforme


Collodion baby

DefinitionNewborn infant enveloped in a shiny,smooth collodion-like membrane, whichmay deform the facial features and distalextremities

ReferencesFrenk E, de Techtermann F (1992) Self-healing

collodion baby: evidence for autosomal reces-sive inheritance. Pediatric Dermatology 9(2):95–97

Colloid degeneration

Synonym(s)Colloid milium; colloid pseudomilium;colloid degeneration of the skin; elastosiscolloidalis conglomerata

Colloid degeneration. Multiple translucent papules on the ear


Coma blister 143

C

DefinitionDeposition of amorphous material (col-loid) in the dermis

PathogenesisRelated to excessive sun exposure; juvenileform inherited; origin of colloid unclear;possibly formed from degeneration of elas-tic fibers or synthesized from ultravioletlight-transformed keratinocytes

Clinical manifestationAdult type: multiple, discrete, shiny, trans-lucent papules in sun-exposed areas of faceand ears; sometimes gelatinous materialextrudedJuvenile type: onset before puberty; numer-ous, yellow-to-brown, waxy papules, mainlyon the face; possibly related to severe sun-burnNodular type: one or a few large, pink-to-brown, smooth nodules on the face

Differential diagnosisNodular amyloidosis; sarcoidosis; epider-moid cyst; syndrome of Favre-Racouchot;sebaceous hyperplasia; xanthoma; tuber-ous sclerosis; porphyria cutanea tarda

TherapyCryotherapy; dermabrasion

ReferencesTouart DM, Sau P (1998) Cutaneous deposition

diseases. Part I. Journal of the American Acad-emy of Dermatology 39(2 Pt 1):149–171

Colloid degeneration of the skin

� Colloid degeneration

Colloid milium


Colloid pseudomilium


Com

� Clavus

Coma blister

Synonym(s)None

DefinitionBullae arising over pressure points inpatients who experience prolonged periodsof unconsciousness

PathogenesisUnclear; several theories proposed: pres-sure necrosis; direct toxic action of a drug,such as a barbiturate or illicit drugs; drug-induced hyperthermia

Clinical manifestationOne or a few vesicles or bullae over pres-sure points, such as fingers, heels, or knees,may involve two limbs apposing oneanother for long periods during an uncon-scious state

Differential diagnosisFixed drug eruption; insect bite reaction;localized bullous pemphigoid; herpes sim-plex virus infection; bullous impetigo; por-phyria cutanea tarda; epidermolysis bul-losa acquisita

TherapyNone

ReferencesMehregan DR, Daoud M, Rogers RS 3rd (1992)

Coma blisters in a patient with diabetic ketoac-


144 Comedone

idosis. Journal of the American Academy of Dermatology 27(2 Pt 1):269–270

Comedone

DefinitionSmall, flesh-colored, white, or dark concre-tion found at the opening of a sebaceousfollicle; also known as whitehead or black-head

ReferencesThiboutot DM (1996) An overview of acne and its

treatment. Cutis 57(1 Suppl):8–12

Còmel-Netherton syndrome

� Netherton syndrome

Common baldness

� Androgenetic alopecia

Common ichthyosis


Compulsive hair pulling


Condyloma acuminata

� Condyloma acuminatum

Condyloma acuminatum

Synonym(s)Genital wart; anogenital wart; condylomaacuminata

DefinitionViral disease characterized by a soft, wart-like growth on the genital skin

PathogenesisHuman papilloma virus (HPV); acquired byinoculation of the virus into the epidermisvia defects in the epithelium or by autoin-oculation

Clinical manifestationPink-to-brown, verrucous, soft papules ornodules of the genitalia, perineum, cruralfolds, and anus, often forming large, exo-phytic, cauliflower-like tumors

Differential diagnosisSyphilis; verrucous carcinoma of genitalia(giant condyloma of Buschke-Löwenstein);bowenoid papulosis; seborrheic keratosis;anogenital carcinoma; erythroplasia ofQueyrat; lichen planus; Reiter syndrome;pearly penile papules

TherapyCryotherapy; imiquimod; podofilox; kera-tolytic agents, such as salicylic acid;destruction by electrodesiccation andcurettage or laser ablation; surgical exci-sion of large tumors

ReferencesKrogh G von (2001) Management of anogenital

warts (condylomata acuminata). European Journal of Dermatology 11(6):598–603

Condyloma lata

DefinitionSkin lesions associated with secondarysyphilis, characterized by flat-topped,


Confluent and reticulated papillomatosis 145

C

necrotic papules clustering in intertrigi-nous sites and secreting a seropurulentfluid

ReferencesRosen T, Hwong H (2001) Pedal interdigital con-

dylomata lata: A rare sign of secondary syphi-lis. Sexually Transmitted Diseases 28(3):184–186

Confluent and reticular papillomatosis


Confluent and reticulate papillomatosis


Confluent and reticulated papillomatosis

Synonym(s)Cutaneous papillomatosis; Gougerot-Car-teaud papillomatosis; Gougerot-Carteaudsyndrome; atrophie brilliante; confluentand reticular papillomatosis; confluent andreticulate papillomatosis; erythrokeratoder-mia papillaris et reticularis; parakeratosebrilliante; pigmented reticular dermatosisof the flexures

DefinitionDisorder characterized by chronic, persist-ent, verrucous papules, with a reticulatedpattern and a tendency to become confluent

PathogenesisPossibly involves abnormal keratinocytedifferentiation and maturation

Clinical manifestationBeginning as small, grayish-brown, hyperk-eratotic papules, enlarging and coalescingto form a reticular pattern peripherally andconfluent plaques centrally; most com-monly occurring on the trunk, face, andneck, and sparing the mucous membranes

Differential diagnosisTinea versicolor; erythrokeratoderma vari-abilis; epidermodysplasia verruciformis;pityriasis rubra pilaris; acanthosis nigri-cans; dermatopathic pigmentosa reticula-ris; dyschromatosis universalis; epidermalnevus; Naegeli-Franceschetti-Jadassohnsyndrome; flat warts

TherapyMinocycline�; isotretinoin; keratolytics;vitamin A; sodium thiosulphate; oral con-traceptives; tretinoin; ultraviolet light; pro-pylene glycol; calcipotriene

ReferencesJang HS, Oh CK, Cha JH, Cho SH, Kwon KS (2001)

Six cases of confluent and reticulated papillo-matosis alleviated by various antibiotics. Jour-nal of the American Academy of Dermatology 44(4):652–655

Confluent and reticulated papillomatosis. Reddish-brown, scaly papules coalescing into reticulated plaques


146 Congenital absence of skin

Congenital absence of skin

� Aplasia cutis congenita

Congenital contractural arachnodactyly syndrome


Congenital dermal melanocytosis

� Mongolian spot

Congenital erythropoietic porphyria

Synonym(s)Gunther's disease; erythropoietic porphy-ria; congenital porphyria; porphyria eryth-ropoietica; congenital hematoporphyria;erythropoietic uroporphyria

DefinitionInborn error of porphyrin-heme synthesisinvolving mutation of a gene encoding theenzyme uroporphyrinogen III synthase,which leads to accumulation of porphyrinsof the isomer I type, that causes cutaneousphotosensitivity

PathogenesisDisorder of bone marrow heme synthesis;deficient uroporphyrinogen III synthaseactivity in erythrocyte precursor cellscauses a shift away from the isomer III por-phyrinogen production that affects the end-

product heme; isomer I porphyrinogens areoverproduced; interaction of excess por-phyrins in the skin and light radiationcauses photo-oxidative damage of biomo-lecular targets, manifested as mechanicalfragility and blistering

Clinical manifestationBlistering and fragility of light-exposedskin; hypertrichosis; teeth have a reddishcolor; blepharitis, cicatricial ectropion, andconjunctivitis; hemolytic anemia can causesecondary hypersplenism

Differential diagnosisErythropoietic protoporphyria; porphyriacutanea tarda; variegate porphyria; pseu-doporphyria; polymorphous light erup-tion; xeroderma pigmentosum; Bloom’ssyndrome

TherapyStrict sun avoidance; erythrocyte transfu-sion; bone marrow transplantation; beta-carotene 120–300 mg PO per day in divideddoses; activated charcoal; cholestyramine;alpha-tocopherol; ascorbic acid

ReferencesDesnick RJ, Astrin KH (2002) Congenital erythro-

poietic porphyria: Advances in pathogenesis and treatment. British Journal of Haematology 117(4):779–795

Congenital erythropoietic protoporphyria

� Erythropoietic protoporphyria

Congenital hematoporphyria

� Congenital erythropoietic porphyria


Congenital self-healing Langerhans cell histiocytosis 147

C

Congenital hemidysplasia

� CHILD syndrome

Congenital histiocytosis X

� Congenital self-healing Langerhans cell histiocytosis

Congenital hypertrichosis

Synonym(s)None

DefinitionExcess hair growth present in the newbornperiod which persists beyond the neonatalperiod

ReferencesSchnur RE (1996) Congenital hypertrichosis. In:

demis DJ (ed) Clinical Dermatology. Lippincott Williams & Wilkins, Philadelphia, Volume 1 Section 2–26

Congenital keratoma of the palms and soles

� Unna-Thost palmoplantar kerato-derma

Congenital, localized absence of skin


Congenital palmoplantar and periorificial keratoderma

� Olmsted Syndrome

Congenital porphyria

� Congenital erythropoietic porphyria� Erythropoietic porphyria

Congenital punctate chondrodystrophy

� Conradi disease

Congenital self-healing Langerhans cell histiocytosis

Synonym(s)Congenital self-healing Langerhans cellreticulohistiocytosis; congenital histiocyto-sis X; Hashimoto-Pritzker disease

DefinitionHeterogeneous eruption, with the histologi-cal appearance of Langerhans cell histiocy-tosis, occurring at birth or in infancy andhealing spontaneously

PathogenesisConsidered a benign variant of Langerhanscell histiocytosis

Clinical manifestationMacules, papules, and nodules of varyingcolor, some hemorrhagic; resolves in 2–3months, sometimes with recurrences; usu-ally no systemic involvement


148 Congenital self-healing Langerhans cell reticulohistiocytosis

Differential diagnosisOther forms of Langerhans cell histiocyto-sis; mastocytosis; lymphoma; juvenile xan-thogranuloma; benign cephalic histiocyto-sis

TherapyNone indicated

ReferencesLarralde M, Rositto A, Giardelli M, Gatti CF, San-

tos Munoz A (1999) Congenital self-healing his-tiocytosis (Hashimoto-Pritzker). International Journal of Dermatology 38(9):693–696

Congenital self-healing Langerhans cell reticulohistiocytosis

� Congenital self-healing Langerhans cell histiocytosis

Congenital telangiectatic erythema

� Bloom’s syndrome

Congenital ulcer of the newborn


Congenital xanthoma tuberosum

� Juvenile xanthogranuloma

Conglobate acne

� Acne conglobata

Conjunctivitis

DefinitionInflammation or infection of the mem-brane lining the eyelids

ReferencesShields SR (2000) Managing eye disease in prima-

ry care. Part 2. How to recognize and treat com-mon eye problems. Postgraduate Medicine 108(5):83–86, 91–96

Connective tissue nevus

Synonym(s)Collagenoma; elastoma; nevus mucinosis

DefinitionHamartomatous proliferation of one ormore connective tissue elements in the der-mis

PathogenesisUnknown

Clinical manifestationMultiple, indurated, cutaneous papules ornodules often over the upper two-thirds ofthe back, associated with multiple endo-crine neoplasia (MEN) type I; shagreenpatch – connective tissue nevus in a patientwith tuberous sclerosis; nevus mucinosis(Hunter syndrome): small, firm papules onthe arms, chest, and over the scapularregion, with coarse facial features, mentalretardation, and deafness

Differential diagnosisMilia; morphea; scar; athlete’s nodules(knuckle pads, etc.); Cowden disease


Contact dermatitis 149

C

TherapySurgical excision for cosmetic reasons only

ReferencesSears JK, Stone MS, Argenyi Z (1988) Papular elas-

torrhexis: A variant of connective tissue nevus. Case reports and review of the literature. Jour-nal of the American Academy of Dermatology 19(2 Pt 2):409–414

Conradi disease

Synonym(s)Conradi Hunermann syndrome; congenitalpunctate chondrodystrophy; chondrodys-trophia calcificans congenita; dysplasia epi-physialis punctata; chondrodysplasia punc-tata, X-linked dominant type

DefinitionForm of chondrodysplasia punctata, char-acterized by punctate opacities within thegrowing ends of long bones and otherregions, dysmorphic facial features, cata-racts, sparse, coarse scalp hair, and/orabnormal thickening, dryness, and scalingof the skin

PathogenesisUnknown; X-linked dominant trait

Clinical manifestationSparse, coarse scalp hair; thickening, dry-ness, and scaling of the skin; mild-to-mod-erate growth deficiency; disproportionateshortening of long bones, particularly thoseof the humeri and the femora; short stat-ure; kyphoscoliosis; prominent foreheadwith midfacial hypoplasia and a low nasalbridge; cataracts

Differential diagnosisEpidermal nevus; incontinentia pigmenti;ichthyosis vulgaris; X-linked ichthyosis

TherapyNone

ReferencesO'Brien TJ (1990) Chondrodysplasia punctata

(Conradi disease). International Journal of Dermatology 29(7):472–476

Conradi Hunermann syndrome

� Conradi disease

Constricting bands of the extremities

� Ainhum

Consumptive thrombocytopenia

� Kasabach-Merritt syndrome

Contact dermatitis

Synonym(s)Dermatitis venenata; contact eczema

DefinitionInflammation of the skin caused by directcontact with an irritating or allergy-caus-ing substance

PathogenesisIrritant variant: caused by direct injury ofthe skin by an agent capable of producing


150 Contact eczema

cell damage in any individual if applied forsufficient time and in sufficient concentra-tionAllergic variant: type IV hypersensitivityreaction only affecting previously sensi-tized individualsContact urticaria variant: possibly immu-nologic in some casesPhotocontact variant: irradiation of certainsubstances by light resulting in the transfor-mation of the substance into full antigens(photoallergic) or irritants (phototoxic)

Clinical manifestationAcute contact stage: red and edematousskin; vesicles or bullae sometimes develop;weeping and oozing as vesicles ruptureSubacute stage: less edematous and ery-thematous; scaling and punctate crustsfrom scratching (excoriations) oftenpresentChronic stage: scaling, fissuring, andlichenification with minimal edemaContact urticaria variant: urticarial whealsat site of contactPhototoxic variant: appearance of an exag-gerated sunburn

Differential diagnosisAtopic dermatitis; dyshidrotic eczema; sun-burn; chemical burn; seborrheic dermati-tis; insect bites; erysipelas; erythema multi-forme; nummular eczema; lichen simplexchronicus; asteatotic eczema; bullous pem-phigoid; pemphigus vulgaris; epidermoly-

sis bullosa; dermatophyte infection; candi-diasis; impetigo; scabies

TherapyRemoval of source of dermatitisMild-to-moderate disease: corticosteroids,topical, mid potency or high potency; alu-minium acetate 5% compresses applied 15–30 minutes 2–4 times dailySevere disease: prednisone�; antihista-mines, first generation, for sedation

ReferencesBruckner AL, Weston WL (2001) Beyond poison

ivy: understanding allergic contact dermatitis in children. Pediatric Annals 30(4):203–206

Moore DE (2002) Drug-induced cutaneous pho-tosensitivity: incidence, mechanism, preven-tion and management. Drug Safety 25(5):345–372

Wakelin SH (2001) Contact urticaria. Clinical & Experimental Dermatology 26(2):132–136

Contact eczema

� Contact dermatitis

Contact stomatitis


Contagious ecthyma

� Orf

Contagious pustular dermatitis

� Orf

Contact dermatitis. Erythematous, edematous plaques around the eyes in a patient with an allergic contact dermatitis to a topical eye medication


Corticosteroids, topical, high potency 151

C

Corn

� Clavus

Cornelia de Lange syndrome

Synonym(s)Brachmann-de Lange syndrome; de Langesyndrome; Amsterdam syndrome; typusdegenerativus amstelodamensis

DefinitionSyndrome characterized by a distinctivefacial appearance, prenatal and postnatalgrowth deficiency, feeding difficulties, psy-chomotor delay, behavioral problems; mal-formations mainly involve the upperextremities

PathogenesisUnknown; few cases transmitted in auto-somal dominant pattern

Clinical manifestationShort stature; microcephaly; facial features:confluent eyebrows, long curly eyelashes,low anterior and posterior hairline, under-developed orbital arches, anteverted nares,down-turned angles of the mouth, thin lips,low-set ears, depressed nasal bridge, micro-gnathia; hypertrichosis; micromelia; behav-ioral abnormalities

Differential diagnosisFetal alcohol syndrome; Coffin-Siris syn-drome


ReferencesOpitz JM, Brachmann-de Lange syndrome (1994)

A continuing enigma. Archives of Pediatrics & Adolescent Medicine 148(11):1206–1208

Corporis circumscriptum naeviforme

� Angiokeratoma circumscriptum

Corpus callosum agenesis-facial anomalies-Robin sequence syndrome

� Toriello-Carey syndrome

Corrugated skin


Corticosteroids, topical, high potency

Trade name(s)Generic in parentheses:Cyclocort (amcinonide); Lidex, Lidex-E,Licon (fluocinonide); Topicort (desoximeta-sone); Diprosone, Maxivate, Alphatrex (bet-amethasone dipropionate); Halog, Halog-E(halcinonide)


Drug classGlucocorticoid

Mechanism of actionAnti-inflammatory; anti-proliferative; atro-phy causing

Dosage formCream; ointment; lotion; gel


152 Corticosteroids, topical, high potency


Common side effectsCutaneous: skin atrophy; steroid addiction(rebound flare after discontinuing the med-ication); tachyphylaxis; increased suscepti-bility to local infection; perioral dermatitis;

delayed wound healing; hypopigmentation;acneform eruption; striae

Serious side effectsMiscellaneous: adrenal insufficiency


Corticosteroids, topical, high potency. Dermatologic indications and dosage


Atopic dermatitis Apply twice daily Apply twice daily

Bullous pemphigoid Apply twice daily Apply twice daily

Contact dermatitis Apply twice daily Apply twice daily

Dyshidrotic eczema; Apply twice daily Apply twice daily

Erythema annulare centrifugum


Follicular mucinosis Apply twice daily Apply twice daily

Herpes gestationis Apply twice daily Apply twice daily

Jessner’s lymphocytic infiltration of skin


Langerhans cell histiocytosis


Lichen planus Apply twice daily Apply twice daily



Lichen striatus Apply twice daily Apply twice daily

Lupus erythematosus, subacute systemic


Nummular eczema Apply twice daily Apply twice daily

Pemphigus vulgaris Apply twice daily Apply twice daily

Pityriasis lichenoides Apply twice daily Apply twice daily

Polymorphous light eruption


Pruritic urticarial papules and plaques of pregnancy



Seabather’s eruption Apply twice daily Apply twice daily

Seborrheic dermatitis Apply twice daily Apply twice daily



T cell lymphoma Apply twice daily Apply twice daily

Xerotic dermatitis Apply twice daily Apply twice daily


Corticosteroids, topical, low potency 153

C

Contraindications/precautionsHypersensitivity to drug class or compo-nent; avoid use on the face for more than 14days; avoid getting in the eye; do not applyin intertriginous areas for more than 1 weekat a time

ReferencesBrazzini B, Pimpinelli N (2002) New and estab-

lished topical corticosteroids in dermatology: clinical pharmacology and therapeutic use. American Journal of Clinical Dermatology 3(1):47–58

Corticosteroids, topical, low potency

Trade name(s)Generic in parentheses:Hydrocortisone 1% (Hytone; Cortef; Cor-taid; Texacort); alclometasone 0.05%(Aclovate); desonide 0.05% (Tridesilon;DesOwen)



Mechanism of actionAnti-inflammatory; antiproliferative; atro-phy-causing

Dosage formCream; ointment; lotion; gel


Common side effectsCutaneous: skin atrophy; steroid addiction(rebound flare after discontinuing the med-ication); tachyphylaxis; increased suscepti-bility to local infection; perioral dermatitis;delayed wound healing; hypopigmentation;acneform eruption; striae



Contraindications/precautionsHypersensitivity to drug class or compo-nent; avoid placing drug in the eye



Corticosteroid, topical, low potency. Dermatologic indications and dosage




Dyshidrosis Apply twice daily Apply twice daily

Netherton syndrome Apply twice daily Apply twice daily


Pityriasis alba Apply twice daily Apply twice daily


Xerotic eczema Apply twice daily Apply twice daily


154 Corticosteroids, topical, medium potency

Corticosteroids, topical, medium potency

Trade name(s)Generic in parentheses:Kenalog, Aristocort (triamcinolone);Valisone, Betatrex, Luxiq (betamethasonevalerate); Cloderm (clocortolone); Cordran(flurandrenolide); Cutivate (fluticasone);Dermatop (prednicarbate); Synalar, Derma-Smoothe (fluocinolone); Elocon (mometa-sone); Locoid (hydrocortisone butyrate);Uticort (betamethasone benzoate); West-cort (hydrocortisone valerate)



Mechanism of actionAnti-inflammatory; anti-proliferative; atro-phy-causing

Dosage formCream; ointment; lotion; gel; foam


Common side effectsCutaneous: skin atrophy; steroid addiction(rebound flare after discontinuing the med-ication); tachyphylaxis; increased suscepti-bility to local infection; perioral dermatitis;delayed wound healing; hypopigmentation;acneform eruption; striae


Corticosteroids, topical, medium potency. Dermatologic indications and dosage



Benign pigmented purpura;


Cercarial dermatitis Apply twice daily Apply twice daily


Dyshidrosis Apply twice daily Apply twice daily

Id reaction Apply twice daily Apply twice daily

Idiopathic guttate hypomelanosis




Pityriasis lichenoides Apply twice daily Apply twice daily

Prurigo of pregnancy Apply twice daily Apply twice daily



Stasis dermatitis Apply twice daily Apply twice daily

Sunburn Apply twice daily Apply twice daily

Wiskott-Aldrich syndrome


Xerotic eczema Apply twice daily Apply twice daily


Coumarin necrosis 155

C


Contraindications/precautionsHypersensitivity to drug class or compo-nent; avoid use on the face for more than 14days; avoid getting in the eye; do not applyin intertriginous areas for more than 2weeks at a time



Corticosteroids, topical, super potency

Trade name(s)Generic in parentheses:Temovate, Olux, Cormax, Embeline(clobetasol); Ultravate (halobetasol); Dipro-lene AF (augmented betamethasone dipro-pionate); Psorcon, Maxiflor, Florone (diflo-rasone diacetate); Cordran Tape (fluran-drenolide tape)



Mechanism of actionAnti-inflammatory; anti-proliferative; atro-phy-causing

Dosage formCream; ointment; lotion; gel; foam; tape


Common side effectsCutaneous: skin atrophy; steroid addiction(rebound flare after discontinuing the med-

ication); tachyphylaxis; increased suscepti-bility to local infection; perioral dermatitis;delayed wound healing; hypopigmentation;acneform eruption; striae



Contraindications/precautionsHypersensitivity to drug class or compo-nent; avoid use on the face; do not apply inintertriginous areas for more than one weekat a time



Coumarin necrosis

Synonym(s)Coumarin skin necrosis; warfarin skinnecrosis

DefinitionRapid onset of localized skin necrosis asso-ciated with recent onset of coumarin ther-apy

PathogenesisLow constitutive levels of protein C; in thepresence of coumarin, levels of protein Cfall more rapidly than do procoagulant fac-tors IX, X and prothrombin, producing atransient hypercoagulable state and localthrombosis of dermal vessels

Clinical manifestationSigns and symptoms beginning 3–5 daysafter initiation of coumarin; single or multi-ple areas of painful erythema rapidly ulcer-


156 Coumarin necrosis

ating and developing a blue-black eschar;most common areas of involvement: thighs,breasts, and buttocks; most likely occur-

ring in patients in whom large initial dosesof coumarin initiated in the absence ofheparin anticoagulation

Corticosteroids, topical, super potency. Dermatologic indications and dosage


Alopecia areata Apply twice daily for up to 2 weeks; 1 week rest period before reuse

Apply twice daily for up to 2 weeks; 1 week rest period before reuse

Atopic dermatitis Apply twice daily for up to 2 weeks; 1 week rest period before reuse


Contact dermatitis Apply twice daily for up to 2 weeks; 1 week rest period before reuse


Dyshidrosis Apply twice daily for up to 2 weeks; 1 week rest period before reuse


Eosinophilic pustular folliculitis



Inflammatory epidermal nevus



Lichen nitidus Apply twice daily for up to 2 weeks; 1 week rest period before reuse


Lichen planus Apply twice daily for up to 2 weeks; 1 week rest period before reuse





Lupus erythematosus, discoid



Lupus erythematosus, subacute systemic



Mastocytosis Apply twice daily for up to 2 weeks; 1 week rest period before reuse


Nummular eczema Apply twice daily for up to 2 weeks; 1 week rest period before reuse


Pemphigus foliaceus Apply twice daily for up to 2 weeks; 1 week rest period before reuse


Pityriasis lichenoides Apply twice daily for up to 2 weeks; 1 week rest period before reuse


Psoriasis Apply twice daily for up to 2 weeks; 1 week rest period before reuse


Reiter syndrome Apply twice daily for up to 2 weeks; 1 week rest period before reuse


Seborrheic dermatitis Apply twice daily for up to 2 weeks; 1 week rest period before reuse


Vitiligo Apply twice daily Apply twice daily

Xerotic eczema Apply twice daily for up to 2 weeks; 1 week rest period before reuse



Cowden/Bannayan-Riley-Ruvalcaba overlap syndrome 157

C

Differential diagnosisOther coagulopathies; heparin necrosis;spider bite reaction; pyoderma gangreno-sum; vasculitis; cutaneous anthrax; trau-matic ulceration; calciphylaxis; necrotizingsoft tissue infection

TherapyMedical therapy: continued coumarin ther-apySurgical therapy: hydrocolloid dressings;skin grafting if healing markedly delayed

ReferencesCole MS, Minifee PK, Wolma FJ (1988) Coumarin

necrosis – A review of the literature. Surgery 103(3):271–277

Coumarin skin necrosis

� Coumarin necrosis

Cowden disease

Synonym(s)Cowden’s syndrome; Cowden syndrome;multiple hamartoma syndrome

DefinitionHamartomatous neoplasms of the skin andmucosa, gastrointestinal tract, bones, cen-tral nervous system, eyes, and genitouri-nary tract

PathogenesisMutation in the PTEN tumor suppressorgene on chromosome 10q23 regulating thefunction of other proteins by removingphosphate groups from those molecules;mutation causing loss of the protein's func-tion and allowing over-proliferation of cells,resulting in hamartomatous growths

Clinical manifestationFlesh-colored, flat-topped, lichenoid orelongated, verrucous papules of the face;

oral cavity papules with a smooth surfaceand a whitish color; sometimes coalescinginto cobblestone-like plaques; acral kera-totic papules, including palmoplantar kera-totic papules; thyroid abnormalities; fibro-cystic disease and fibroadenomas of thebreast; increased incidence of breast carci-noma; gastrointestinal polyps; ovariancysts; uterine leiomyomas

Differential diagnosisWart; sebaceous hyperplasia; milia; xan-thoma; trichilemmoma; trichoepithelioma;Darier disease; syringoma; fibrofollicu-loma; multiple benign fibromas; multipleendocrine neoplasia; tuberous sclerosis;lipoid proteinosis; Goltz syndrome; floridoral papillomatosis

TherapySurgical therapy: chemical peel; laser resur-facing; excisional surgery

ReferencesHildenbrand C, Burgdorf WH, Lautenschlager S

(2001) Cowden syndrome-Diagnostic skin signs. Dermatology 202(4):362–366

Cowden syndrome

� Cowden disease

Cowden’s syndrome

� Cowden disease

Cowden/Bannayan-Riley-Ruvalcaba overlap syndrome

� Bannayan-Riley-Ruvalcaba syn-drome


158 Creeping eruption

Creeping eruption

� Cutaneous larva migrans

Crocker syndrome

� Niemann-Pick disease

Crocker's syndrome


Crocker-Farber syndrome


Cronkhite Canada syndrome

Synonym(s)Gastrointestinal polyposis syndrome, gen-eralized, associated with hyperpigmenta-tion, alopecia, and nail atrophy

DefinitionAssociation of generalized gastrointestinalpolyps, cutaneous pigmentation, alopecia,and onychodystrophy

PathogenesisUnknown

Clinical manifestationOnset of constant or episodic pain in thelower or upper abdomen, with weight loss;alopecia simultaneously from the scalp, eye-brows, face, axillae, pubic areas, andextremities; lentigo-like macules and/or dif-

fuse hyperpigmentation, including the buc-cal mucosa; nail dystrophy; peripheral orgeneralized edema; multiple gastrointesti-nal polyps, with increased incidence ofcolon carcinoma

Differential diagnosisGardner’s syndrome; Peutz-Jeghers syn-drome; Bandler syndrome; Ménétrier dis-ease; familial polyposis

TherapyNo therapy for cutaneous manifestations;close follow-up for gastrointestinal prob-lems

ReferencesFinan MC, Ray MK (1989) Gastrointestinal poly-

posis syndromes. Dermatologic Clinics 7(3):419–434

Crotch rot

� Tinea cruris

Crow-Fukase syndrome

� POEMS Syndrome

Cryofibrinogenemia

Synonym(s)None

DefinitionPresence of the cryoprotein, cryofibrino-gen, in serum, with resultant cutaneousmanifestations

PathogenesisUnknown


Cryptococcosis 159

C

Clinical manifestationPrimary (essential) form: unassociated withunderlying disease; secondary form: associ-ated most commonly with internal malig-nancies and thromboembolic disease, butalso with rheumatic diseases, diabetes mel-litus, and pregnancy; purpura; ecchy-moses; cutaneous gangrene; persistent,painful ulcerations, with surroundinglivedo reticularis

Differential diagnosisCryoglobulinemia; benign pigmented pur-pura; antiphospholipid antibody syn-drome; Churg-Strauss syndrome; polyar-teritis nodosa; serum sickness; Walden-ström hyperglobulinemia; septic vasculitis;systemic lupus erythematosus; sarcoidosis

TherapyStanozolol: 4–8 mg PO daily; plasmapher-esis

ReferencesHelfman T, Falanga V (1995) Stanozolol as a novel

therapeutic agent in dermatology. Journal of the American Academy of Dermatology 33(2 Pt 1):254–258

Cryoglobulinemia

Synonym(s)Cryoproteinemia

DefinitionPresence of abnormal proteins in the blood-stream, which thicken or gel on exposure tocold

PathogenesisSome of the sequelae of cryoglobulinemiarelated to immune-complex disease; othersequelae related to cryoprecipitation invivo, including plugging and thrombosis ofsmall arteries and capillaries; some cases inotherwise normal patients (essential mixedcryoglobulinemia)

Clinical manifestationSkin findings: palpable purpura; distalnecrosis; urticaria, and ischemic necrosisleading to ulceration; cold-induced urti-caria; acrocyanosisInternal manifestations: pulmonary; renal;joints; central nervous system; sometimespresent in mycoplasma pneumonia, viralhepatitis, multiple myeloma, certain leuke-mias, primary macroglobulinemia, andsome autoimmune diseases, such as sys-temic lupus erythematosus and rheuma-toid arthritis

Differential diagnosisAntiphospholipid antibody syndrome;Churg-Strauss syndrome; polyarteritisnodosa; serum sickness; Waldenströmhyperglobulinemia; septic vasculitis; sys-temic lupus erythematosus; sarcoidosis

TherapyNo therapy indicated for asymptomatic dis-easeSymptomatic disease: nonsteroidal anti-inflammatory drugs; prednisoneSteroid-sparing medications: azathioprine;cyclophosphamidePlasmapheresis for life-threatening disease;interferon-α for cryoglobulinemia associ-ated with hepatitis C infection

ReferencesCacoub P, Costedoat-Chalumeau N, Lidove O, Al-

ric L (2002) Cryoglobulinemia vasculitis. Cur-rent Opinion in Rheumatology 14(1):29–35

Cryoproteinemia

� Cryoglobulinemia

Cryptococcosis

Synonym(s)Busse-Buschke disease; European blasto-mycosis; torulosis


160 Cushing syndrome

DefinitionFungal infection caused by the inhalation ofthe fungus, Cryptococcus neoformans

PathogenesisHuman disease associated only with Cryp-tococcus neoformans; following inhalationof the organism, alveolar macrophagesingest the yeast; cryptococcal polysaccha-ride capsule has antiphagocytic propertiesand may be immunosuppressive; anti-phagocytic properties of the capsule blockrecognition of the yeast by phagocytes andinhibit leukocyte migration into the area offungal replication; decreased host immu-nity main element in susceptibility to clini-cal infection; organ damage primarily fromtissue distortion secondary to increasingfungal burden

Clinical manifestationPre-existing medical problems, such as sys-temic steroid use, malignant disease, organtransplantation, or HIV infection.Skin findings: papules, sometimes umbili-cated; pustules; nodules; ulcers; drainingsinuses; rarely occurs as a primary inocula-tion diseaseInternal organ involvement: pulmonary –variable, ranging from asymptomatic air-way colonization to acute respiratory dis-tress syndromeCentral nervous system: usually meningitisor meningoencephalitis

Differential diagnosisPyogenic abscess; nocardia, aspergillosis;lymphoma; meningeal metastases; tubercu-losis; histoplasmosis; acne; molluscum con-tagiosum; syphilis; toxoplasmosis

TherapyNon-AIDS-related: amphotericin B 0.5–1 mg per kg per day IV; total cumulativedose of 3 gm�; fluconazoleAIDS-related infection: initially, amphoter-icin B for 2 weeks, with or without 2 weeksof flucytosine, followed by fluconazole for aminimum of 10 weeks�

ReferencesThomas I, Schwartz RA (2001) Cutaneous mani-

festations of systemic cryptococcosis in immu-nosuppressed patients. Journal of Medicine 32(5-6):259–266

Cushing syndrome

Synonym(s)Hypercorticalism; Cushing’s syndrome

DefinitionHormonal disorder caused by prolongedexposure of the body's tissues to high levelsof cortisol

PathogenesisExcess levels of either exogenously adminis-trated glucocorticoids or endogenous over-production of cortisol from tumors or adre-nal gland hyperplasia, lead to signs andsymptoms of hypercorticalism

Clinical manifestationSkin changes: facial plethora; striae; ecchy-moses and purpura; telangiectasias; skinatrophy; hirsutism and male pattern bald-ing in women; increased lanugo facial hair;steroid acne; acanthosis nigricansCentral obesity; increased adipose tissue inthe face (moon facies), upper back at thebase of neck (buffalo hump), and above theclaviclesEndocrine abnormalities: hypothyroidism;galactorrhea; polyuria and nocturia fromdiabetes insipidusMenstrual irregularities, amenorrhea, andinfertilityOther organ system abnormalities: cardio-vascular; musculoskeletal; gastroentero-logic; neuropsychological

Differential diagnosisExogenous obesity; anorexia nervosa; alco-holism; drug effects from phenobarbitalphenytoin or rifampin; psychiatric illness


Cutaneous CD30+ (Ki-1) anaplastic large-cell lymphoma 161

C

TherapyMedical therapy: ketoconazole�

Surgical therapy: surgical resection of thecausative tumor, if present, either by trans-sphenoidal surgery for pituitary tumors oradrenalectomy for adrenal tumors�; pitui-tary irradiation when transsphenoidal sur-gery not successful or not possible

ReferencesNorton JA, Li M. Gillary J, Le HN (2001) Cushing's

syndrome. Current Problems in Surgery 38(7):488–545

Cushing’s syndrome

� Cushing syndrome

Cutaneomeningospinal angiomatosis

� Cobb syndrome

Cutaneous aspergillosis

Synonym(s)None

DefinitionCutaneous manifestation of disseminatedinfection with the fungus Aspergillus

PathogenesisCaused by infection with soil- and water-dwelling saprophytes of the Aspergillusgenus; initial infection of the pulmonarysystem via inhalation of fungal spores;hematogenous dissemination leads to skininvolvement

Clinical manifestationBegins as a febrile illness, pneumonia, orsinusitis; asymptomatic or tender, solitaryor multiple, erythematous or violaceousindurated papules or plaques, sometimes atthe sites of an intravenous catheter or avenipuncture; rapid evolution into pustulesand hemorrhagic vesicles, producingeschars

Differential diagnosisEcthyma; mucormycosis; cryptococcosis;phaeohyphomycosis; ecthyma gangreno-sum; Sweet’s syndrome; pyoderma gan-grenosum

TherapyAmphotericin B 3–5 mg per kg per dayintravenously, increasing dose as tolerat-ed�; itraconazole

Referencesvan Burik JA, Colven R, Spach DH (1998) Cutane-

ous aspergillosis. Journal of Clinical Microbiol-ogy 36(11):3115–3121

Cutaneous calcinosis

� Calcinosis cutis

Cutaneous calculi

� Calcinosis cutis

Cutaneous CD30+ (Ki-1) anaplastic large-cell lymphoma

Synonym(s)Regressing atypical histiocytosis; CD30+cutaneous large T-cell lymphoma, pseudo-Hodgkin disease


162 Cutaneous ciliated cyst

DefinitionHeterogeneous neoplastic disorder, charac-terized by either primary cutaneous formwithout extracutaneous involvement or sys-temic form with secondary skin involve-ment at onset of disease activity

PathogenesisNeoplastic cells are CD30 positive and usu-ally have T-helper phenotype; systemicform related to novel fusion protein (NPM-ALK)

Clinical manifestationPrimary cutaneous form: solitary or few,reddish-brown, indurated, ulcerative nod-ules or tumors; sometimes spontaneouslyregressing; involvement of drainingregional lymph nodes; good prognosisSystemic form: skin and systemic lesions atpresentation; poor prognosis

Differential diagnosisLymphomatoid papulosis; CD30 negativelymphoma; Hodgkin’s disease; Jessner’sbenign lymphocytic infiltration; granu-loma faciale; metastasis; Merkel cell carci-noma; melanoma; squamous cell carci-noma; basal cell carcinoma

TherapySolitary or localized cutaneous disease:radiation therapy�; methotrexate; surgicalexcisionSystemic disease: multidrug cancer chemo-therapy

ReferencesLeBoit PE (1996) Lymphomatoid papulosis and

cutaneous CD30+ lymphoma. American Jour-nal of Dermatopathology 18(3):221–23

Cutaneous ciliated cyst

� Cutaneous columnar cyst

Cutaneous columnar cyst

Synonym(s)Cutaneous ciliated cyst

DefinitionDevelopmental cyst with columnar epithe-lial lining

PathogenesisDerived from embryological vestiges, suchas the branchial arch cleft, thyroglossalduct, tracheobronchial bud, urogenitalsinus, and Müllerian structures; representsincomplete involution of embryologic ves-tigial structures

Clinical manifestationThyroglossal cyst: occurring anywherealong thyroglossal duct, from base oftongue to the anterior neck; asymptomatic,gradually enlarging, near-midline nodulethat moves with swallowing; drainage ofclear or purulent fluidThymic cyst: found in the mediastinum orneck; ill-defined painless swelling in chil-drenBronchogenic cyst: present at birth or inneonatal period in suprasternal notch,neck, scapular area, and chin; sometimesforming sinuses and drains mucoid fluidCutaneous ciliated cyst: occurs primarily onthe leg in women; ill-defined subcutaneousswelling without central poreMedian raphe cyst: midline developmentalcyst on ventral penis or scrotum, on rapheconnecting external urethral meatus toanus

Differential diagnosisBenign tumor of adnexal structure; lipoma;epidermoid cyst; dermoid cyst; eruptivevellus hair cyst; basal cell carcinoma;melanocytic nevus; steatocystoma multi-plex


Cutaneous larva migrans 163

C


ReferencesEnepekides DJ (2001) Management of congenital

anomalies of the neck. Facial Plastic Surgery Clinics of North America 9(1):131–145

Cutaneous horn

DefinitionConical projection above the surface of theskin, resembling a miniature animal horn,occurring in conjunction with underlyingdermatoses such as wart, actinic keratosis,seborrheic keratosis, basal cell carcinoma,squamous cell carcinoma, and keratoacan-thoma

ReferencesThappa DM, Garg BR, Thadeus J, Ratnakar C

(1997) Cutaneous horn: A brief review and re-port of a case. Journal of Dermatology 24(1):34–37


Synonym(s)Creeping eruption; larva migrans;plumber's itch; sandworm disease

DefinitionDisorder characterized by percutaneouspenetration and subsequent migration oflarvae of various nematode parasites

PathogenesisAncylostoma braziliense (hookworm ofwild and domestic dogs and cats) mostcommon cause; in humans (accidentalhosts), larvae lack enzymes required toinvade through the dermis, so disease lim-ited to the skin

Clinical manifestationOften associated with history of sunbath-ing or walking barefoot on the beach; tin-gling/prickling, pruritus at site of exposurewithin 30 minutes of larvae penetration;advancing, erythematous, often linearlesions, occurring on dorsa of feet, interdig-ital spaces of toes, anogenital region, but-tocks, hands, and knees; 2–3-mm-wide, ser-piginous, slightly elevated, erythematoustunnels, tracking 3–4 cm from penetrationsite; vesicles with serous fluid; occasionalsecondary impetiginization; systemic signs:peripheral eosinophilia and increasedIgE levels

Differential diagnosisScabies; insect bite reaction; foreign bodygranuloma; dermatophytosis; erythemamigrans; myiasis; photoallergic dermatitis;larva currens

Cutaneous horn. Keratotic horn arising from the center of a papule on the upper extremity

Cutaneous larva migrans. Serpiginous, linear, red-brown plaque on the foot


164 Cutaneous lymphangioma

TherapyThiabendazole: 10–15% suspension underocclusive dressing 4 times daily for 1 weekor 25–50 mg PO every 12 hours for 2–5 days�; albendazole; ivermectin

ReferencesCaumes E (2000) Treatment of cutaneous larva

migrans. Clinical Infectious Diseases 30(5):811–814

Cutaneous lymphangioma

� Lymphangioma

Cutaneous lymphomatous hyperplasia

� Pseudolymphoma

Cutaneous lymphoplasia

� Pseudolymphoma

Cutaneous papillomatosis


Cutaneous periarteritis nodosa

� Polyarteritis nodosa

Cutaneous strongyloidiasis

� Strongyloidosis

Cutaneous TB


Cutaneous tuberculosis

Synonym(s)Cutaneous TB; tuberculous chancre;tuberculosis verrucosa cutis; miliary tuber-culosis of the skin; scrofuloderma;tuberculous gumma; tuberculosis cutis ori-ficialis; lupus vulgaris; lichen scrofu-losorum

DefinitionCutaneous manifestations of an airbornecommunicable disease that occurs afterinhalation of infectious droplets expelledfrom patients with laryngeal or pulmonaryTB

PathogenesisSystemic spread of a pulmonary infection,often in a host with poor immunity; directinnoculation into the skin of the tuberculebacillus

Clinical manifestationPrimary inoculation TB (tuberculous chan-cre): chronic, shallow, nontender, under-mined ulcer; painless regional lymphaden-opathy TB verrucosa cutis: slow growing verru-cous papule; may show central involutionwith an atrophic scar; fissures with puru-lent and keratinous materialMiliary TB of the skin: small red macules orpapules with purpura, vesicles, and central


Cutis laxa 165

C

necrosis in a patient with fulminant tuber-culosisScrofuloderma: firm, painless, subcutane-ous nodules that enlarge and suppurate,forming ulcers and sinus tracts in overly-ing skinTB cutis orificialis: affects orificial sitessuch as tip and lateral margins of thetongue, hard and soft palate, perianal skin,the vulva, the urinary meatus, and the glanspenis; lesions present as red papules thatevolve into painful, soft, punched-out, shal-low ulcersLupus vulgaris: solitary, small, sharply mar-ginated, red-brown papules of the head andneck, which slowly evolve by peripheralextension and central atrophy into largeplaquesLichen scrofulosorum: asymptomatic,grouped, closely set, small, perifollicular,lichenoid papules; occur in children andyoung adults with TB

Differential diagnosisSarcoidosis; disseminated deep fungalinfection; sporotrichosis; squamous cellcarcinoma; pyoderma gangrenosum; lym-phoma; pseudolymphoma; leprosy; leish-maniasis; syphilis; actinomycosis;tularemia; Langerhans cell histiocytosis

TherapyFirst 2 months of therapy: isoniazid 5 mgper kg per day in adults; 10–20 mg per kgper day in children; rifampin 10 mg per kgper day in adults; 10–20 mg per kg per dayin children; pyrazinamide 15–30 mg per kgper day in adults and children; ethambutol15–25 mg per kg per day in adults and chil-dren or streptomycin 15 mg per kg per dayin adults; 20–40 mg per kg per day in chil-drenNext 4 months of therapy: isoniazid andrifampin if isolates are sensitive

ReferencesSmall PM, Fujiwara PI (2001) Management of tu-

berculosis in the United States. New England Journal of Medicine 345(3):189–200

Cutis hyperelastica

� Ehlers Danlos syndrome

Cutis laxa

Synonym(s)Cutis pendula; dermatochalasis; elastolysis;dermatomegaly; elastolysis cutis laxa

DefinitionConnective tissue disorder in which skinloses its elasticity and hangs in folds

PathogenesisPossibly due to abnormal elastin metabo-lism, resulting in markedly reduced dermalelastin content and degenerative changes inelastic fibers; biochemical basis of the dis-order may be heterogeneous

Clinical manifestationSkin loose, inelastic, hanging in folds, anddemonstrating decreased elastic recoil onstretching; patient looks much older thanchronologic ageInternal organ involvement: gastrointesti-nal tract: diverticula of small and largebowel; rectal prolapse; umbilical, inguinal,and hiatal herniasPulmonary: bronchiectasis, emphysema,cor pulmonaleCardiovascular: cardiomegaly; congestiveheart failure; murmurs; aortic aneurysmsSkeletal: dislocation of hips; osteoporosis;growth retardation; delayed fontanelle clo-sure; ligamentous laxity

Differential diagnosisCostello syndrome; Ehlers-Danlos syn-drome; granulomatous slack skin variant ofperipheral T cell lymphoma; Marfan syn-drome; mid-dermal elastolysis; pseudoxan-thoma elasticum; anetoderma; atrophode-rma of Pasini and Pierini


166 Cutis pendula


ReferencesDeAngelis DD, Carter SR, Seiff SR (2002) Derma-

tochalasis. International Ophthalmology Clin-ics 42(2):89–101

Cutis pendula

� Cutis laxa

Cutis rhomboidalis

DefinitionDeep furrows in a rhomboid geometric pat-tern on the posterior neck, as a sign ofadvanced sun damage

ReferencesGoldberg LH, Altman A (1984) Benign skin

changes associated with chronic sunlight expo-sure. Cutis 34(1):33–38,40

Cutis sulcata


Cutis verticis gyrata

Synonym(s)Robert-Unna syndrome; bulldog scalp;cutis sulcata; corrugated skin; cutis verticisplicata; pachydermia verticis gyrata

DefinitionScalp condition characterized by convo-luted folds and furrows formed by thick-ened skin

PathogenesisPrimary form: unknown etiology; possiblefactor, is increased peripheral use of testo-sterone.Secondary form: depends on the underly-ing process (e.g. systemic diseases, inflam-matory dermatoses, underlying nevoidabnormalities, and trauma)

Clinical manifestationPrimary form: only scalp involvement; sym-metrical, soft, and spongy folds developingafter puberty, usually in vertex and occipi-tal regionSecondary form: sometimes present at birthBoth forms: hair over the folds sometimessparse but normal in the furrows; macera-tion and unpleasant smell sometimespresent in cases with secondary infection inthe furrows

Differential diagnosisAcromegaly; cutis laxa; pachydermoperios-tosis; congenital nevus; cylindroma

TherapySurgical resection for psychological oresthetic reasons

ReferencesSnyder MC, Johnson PJ, Hollins RR (2002) Con-

genital primary cutis verticis gyrata. Plastic & Reconstructive Surgery 110(3):818–821

Cutis verticis gyrata. Soft, spongy folds of skin on the posterior scalp


Cyclosporine 167

C

Cutis verticis plicata


Cyclophosphamide

Trade name(s)Cytoxan; Neosar

Generic availableNo

Drug classAlkylating agent; immunosuppressant

Mechanism of actionCell-cycle nonspecific suppression of B cellsand T cells; forms DNA cross-links

Dosage form25 mg, 50 mg tablets; 100 mg, 200 mg,300 mg vials for intravenous injection


Common side effectsCutaneous: alopecia, stomatitis, dyspig-mentation of skin and nails, skin eruptionGastrointestinal: nausea and vomiting,diarrheaGenitourinary: cystitis

Serious side effectsBone marrow: suppressionCardiovascular: congestive failure, cardio-myopathyCutaneous: anaphylaxisGenitourinary: hemorrhagic cystitis, steril-ity; increased risk of cancer

Drug interactionsBone marrow suppressants; allopurinol;doxorubicin; zidovudine

Contraindications/precautionsHypersensitivity to drug class or compo-nent; bone marrow depression; caution inimpaired renal or liver function; caution inleukopenia or thrombocytopenia


drugs. Dermatologic Clinics 19(1):105– 118

Cyclosporine

Trade name(s)Neoral; Sandimmune; SangCya


Drug classImmunosuppressive

Mechanism of actionCalcineurin inhibition causes decreased IL-2 production; leads to decline in activated Tlymphocytes

Dosage formNeoral: 25 mg, 100 mg capsule; 100 mg perml oral solution.Sandimmune: 25 mg, 50 mg, 100 mg cap-sule; 100 mg per ml oral solution; 50 mg perml for IV infusion


Common side effectsCutaneous: hypertrichosis, acne, gingivalhyperplasiaGastrointestinal: nausea and vomiting,diarrhea, abdominal painLaboratory: elevated liver function tests,elevated BUN and creatinine, hyperkale-mia, hyperuricemia, hypomagnesemiahyperglycemia


168 Cyclosporine

Serious side effectsBone marrow: suppressionCutaneous: anaphylaxisNeurologic: seizuresRenal: nephrotoxicity

Drug interactionsAntifungal agents; barbiturates; car-bamazepine; carboplatin; cimetidine; cipro-

floxacin; colchicine; oral contraceptives;diltiazem; systemic corticosteroids; eryth-romycin; lovastatin; glyburide/metformin;metronidazole; nafcillin; non-steroidal anti-inflammatory agents; phenytoin; pimozide;potassium salts; pravastatin; protease inhib-itors; rifampin; simvastatin; verapamil;vinca alkaloids

Cyclophosphamide. Dermatologic indications and dosage



50–200 mg PO daily Not indicated

Behçet’s disease 50–200 mg PO daily Not indicated

Bullous pemphigoid 50–200 mg PO daily Not indicated



Cryoglobulinemia 50–200 mg PO daily Not indicated

Dermatomyositis 50–200 mg PO daily Not indicated



Fogo selvagem 50–200 mg PO daily Not indicated

Lichen myxedematosus


Lupus erythematosus 50–200 mg PO daily Not indicated





Pemphigus foliaceus 50–200 mg PO daily Not indicated

Pemphigus vulgaris 50–200 mg PO daily Not indicated

Polyarteritis nodosa 50–200 mg PO daily Not indicated





Scleroderma 50–200 mg PO daily Not indicated

Vasculitis 50–200 mg PO daily Not indicated




2 mg per kg PO daily or 0.5 g per m2 IV every month for 6 months

Not indicated


Cyclosporine 169

C

Cyclosporine. Dermatologic indications and dosage


Alopecia areata 3–5 mg per kg PO daily, divided into 2 doses

3–5 mg per kg PO daily, divided into 2 doses

Atopic dermatitis 3–5 mg per kg PO daily, divided into 2 doses


Behçet’s disease 3–5 mg per kg PO daily, divided into 2 doses


Bullous pemphigoid 3–5 mg per kg PO daily, divided into 2 doses





Dermatomyositis 3–5 mg per kg PO daily, divided into 2 doses





Hyperimmunoglobulin E syndrome

3 mg per kg daily for 6 months 3 mg per kg daily for 6 months

Lichen amyloidosis 3–5 mg per kg PO daily, divided into 2 doses

Not indicated

Lichen planus, erosive Oral solution applied to erosions 3–4 times daily

Oral solution applied to erosions 3–4 times daily




Mycosis fungoides 3–5 mg per kg PO daily, divided into 2 doses


Pemphigus vulgaris 3–5 mg per kg PO daily, divided into 2 doses


Pityriasis rubra pilaris 3–5 mg per kg PO daily, divided into 2 doses


Psoriasis 3–5 mg per kg PO daily, divided into 2 doses

Not indicated




Reiter syndrome 3–5 mg per kg PO daily, divided into 2 doses

Not indicated




Scleroderma 3–5 mg per kg PO daily, divided into 2 doses

Not indicated

Sézary’s syndrome 3–5 mg per kg PO daily, divided into 2 doses


Urticaria 3–5 mg per kg PO daily for no longer than 3 months; to be used only for severe, recalcitrant disease

3–5 mg per kg PO daily for no longer than 3 months; to be used only for severe, recalcitrant disease


170 Cylindroma

Contraindications/precautionsHypersensitivity to drug class or compo-nent; caution with impaired renal orhepatic function; caution with other poten-tially nephrotoxic drugs

ReferencesCather J, Abramovits W, Menter A (2000) Cy-

closporine and tacrolimus in dermatology. Dermatologic Clinics 19(1);119–138

Cylindroma

Synonym(s)Turban tumor; tomato tumor

DefinitionPrimitive, benign, sweat gland tumor, mostcommonly occurring on the head, neck,and scalp

PathogenesisSolitary tumor variant: unknown; tumordifferentiation toward either the eccrine orapocrine lineMultiple tumor variant: autosomal domi-nant trait

Clinical manifestationSolitary tumor variant: firm, rubbery, red-to-blue papule or nodule, located on scalp,head, or neck; rare malignant transforma-tionMultiple tumor variant: numerous massesof pink, red, or blue papules or nodules,sometimes resembling bunches of smalltomatoes; located on the head and neckregion, trunk, or extremities

Differential diagnosisPilar cyst; eccrine spiradenoma; metas-tases; cutis verticis gyrata

TherapySolitary or multiple small tumors: simpleexcision or CO2 laser ablationMultiple clustered tumors: extensive exci-sions with reconstruction

ReferencesGerretsen AL, van der Putte SC, Deenstra W, van

Vloten WA (1993) Cutaneous cylindroma with malignant transformation. Cancer 72(5):1618–1623

Cyst

Synonym(s)None

DefinitionA sac or capsule filled with fluid, muci-nous, or keratinous material

ReferencesLangley RG, Walsh N, Ross JB (1997) Multiple

eruptive milia: report of a case, review of the literature, and a classification. Journal of the American Academy of Dermatology 37(2 Pt 2):353–356

Cyst, dermoid

� Dermoid cyst



Not indicated

Cyclosporine. Dermatologic indications and dosage (Continued)



Cysticercosis 171

C

Cyst, epidermoid

� Epidermoid cyst

Cyst, mucinous

� Digital mucous cyst

Cyst, myxoid


Cystadenoma, apocrine


Cystic chromomycosis


Cystic hidradenoma


Cystic hygroma

� Lymphangioma

Cysticercosis

Synonym(s)Neurocysticercosis; Taenia solium infesta-tion

DefinitionSystemic illness caused by dissemination ofthe larval form of the pork tapeworm, Tae-nia solium

PathogenesisIntermediate host (normally pigs) ingestseggs in contaminated food or water; Tsolium embryos penetrate GI mucosa of thepig and are hematogenously disseminatedto peripheral tissues, with formation of lar-val cysts (cysticerci); with consumption ofundercooked pork, intestinal tapewormagain formed, completing the life cycle ofthe worm; cyst dissemination in humanscause signs and symptoms of disease

Clinical manifestationSkin findings: subcutaneous nodulesresembling epidermoid cystsNeurologic findings: papilledema anddecreased retinal venous pulsations; men-ingismus; hyperreflexia; nystagmus or vis-ual deficitsMusculoskeletal findings: muscular pseudo-hypertrophy

Differential diagnosisToxoplasmosis; coccidioidomycosis; tuber-culosis; meningitis; encephalitis; brainabscess; cerebrovascular accident; sarcoido-sis; brain tumor

TherapyAlbendazole 15 mg per kg per day POdivided into 2 or 3 doses for 2 weeks; prazi-quantel 50 mg per kg per day PO dividedinto 3 doses for 2 weeks

ReferencesGarcia HH, Del Brutto OH (2000) Taenia solium

cysticercosis. Infectious Disease Clinics of North America 14(1):97–119


172 Cystomata

Cystomata


Cytophagic histiocytic panniculitis

Synonym(s)None

DefinitionProliferative disorder of histiocytes, charac-terized by fever, subcutaneous nodules, andabnormal liver function

PathogenesisUnknown

Clinical manifestationTender, red, subcutaneous nodules, some-times ulcerating; mucous membrane ulcer-ations; enlarged liver and spleen; lymphad-enopathy; prolonged clinical course, usu-ally ending with pancytopenia andhepatosplenomegaly

Differential diagnosisWeber-Christian disease; lymphoma; nodu-lar vasculitis; polyarteritis nodosa; lupusprofundus; traumatic panniculitis; pancre-atic panniculitis; alpha-1 anti-trypsin defi-ciency; factitial disease; pyoderma gan-grenosum; Sweet’s syndrome

TherapyPrednisone; cyclosporine

ReferencesRequena L, Sanchez Yus E (2001) Panniculitis.

Part II. Mostly lobular panniculitis. Journal of the American Academy of Dermatology 45(3):325–361


D

Dabska tumor

�

Endovascular papillary angioen-dothelioma of childhood

Dactylitis

Definition

Inflammation of the fingers and/or toes

References

Rhody C (2000) Bacterial infections of the skin. Primary Care: clinics in Office Practice 27(2):459–473

Dactylolysis spontanea

�

Ainhum

Danbolt-Closs syndrome

�


Dandruff

�

Seborrheic dermatitis

Dapsone

Trade name(s)

None

Generic available

Yes

Drug class

Sulfone

Mechanism of action

Leprosy: folic acid pathway inhibitionInflammatory disorders: effects on neu-trophils, including inhibition of myeloper-oxidase and inhibition of neutrophil chem-otaxis

Dosage form

25 mg, 100 mg tablet


See table

Common side effects

Cutaneous:

skin eruption, including urti-caria; photosensitivity


174 Dapsone

Dapsone. Dermatologic indications and dosage


Acropustulosis of infancy

Start at 100 mg PO daily; titrate as per therapeutic response

2 mg per kg PO daily




Behçet’s disease Start at 100 mg PO daily; titrate as per therapeutic response





Bullous pemphigoid Start at 100 mg PO daily; titrate as per therapeutic response





Dermatitis herpetiformis



Eosinophilic pustular folliculitis and other forms of folliculitis






Erythema elevatum diutinum



Eumycetoma 50–200 mg PO daily for months to years

25–50 mg PO daily for months to years

Granuloma annulare Start at 100 mg PO daily; titrate as per therapeutic response


Granuloma faciale Start at 100 mg PO daily; titrate as per therapeutic response





Leprosy Start at 100 mg PO daily; titrate as per therapeutic response


Linear IgA dermatosis 25–100 mg PO daily initially; if blistering is not controlled, use 50 mg increments every 1–2 weeks

1–2 mg per kg PO daily

Lupus erythematosus, bullous



Pemphigus foliaceus Start at 100 mg PO daily; titrate as per therapeutic response


Pemphigus vulgaris Start at 100 mg PO daily; titrate as per therapeutic response






Darier disease 175

D

Gastrointestinal:

nausea, vomiting, abdom-inal pain, pancreatitis

General:

malaise

Neurologic:

dizziness, peripheral neuropa-thy


Cutaneous:

dapsone hypersensitivity syn-drome, exfoliative dermatitis, toxic epider-mal necrolysis

Gastrointestinal:

hepatotoxicity

Hematologic:

agranulocytosis; leukopenia;methemoglobinemia

Renal:

acute tubular necrosis

Drug interactions

Antacids; bone marrow suppressants; cloza-pine; cytotoxic chemotherapeutic agents;interferon; probenecid; trimethoprim; zido-vudine


Hypersensitivity to drug class or compo-nent; caution in G6PD deficiency, impairedrenal function, or decreased liver function

References

Paniker U, Levine N (2000) Dapsone and sulfapy-ridine. Dermatologic Clinics 19(1):79–86

Darier disease

Synonym(s)

Darier’s disease

;

Darier-White disease

;

keratosis follicularis

Definition

Dominantly inherited disease character-ized by hyperkeratotic papules in sebor-rheic regions and nail abnormalities

Pathogenesis

Abnormal cell-cell adhesion and aberrantepidermal keratinization; mutations in thegene ATP2A2, which encodes a calciumpump; calcium-dependent signaling path-way in desmosomal assembly and cell-celladhesion; defects cause alterations ofcytosolic calcium level, influencing adhe-sion between keratinocytes and cellular dif-ferentiation in the epidermis


Yellowish-brown, greasy, verrucouspapules, most common in the seborrheicareas, such as forehead, scalp, nasolabialfolds, ears, chest and back; mucosal sur-faces with white papules with central




Subacute nodular migratory panniculitis






Urticaria 50–100 mg PO daily for no longer than 3 months; to be used only for severe, recalcitrant disease

Not indicated

Vasculitis, including urticarial vasculitis



Dapsone. Dermatologic indications and dosage (Continued)



176 Darier-White disease

depression; heat, humidity, stress, sunlight,and UVB rays exacerbate the condition;lesions on palms, including punctate kera-tosis, palmar pits, and hemorrhagic mac-ules; verrucous papules present on thebacks of the hands; nail changes, includingwhite and red longitudinal bands, longitu-dinal nail ridges, and splits


Transient acantholytic dermatosis (Grover’sdisease); Hailey-Hailey disease; pemphigusfoliaceus; seborrheic dermatitis; acrokera-tosis verruciformis of Hopf; pityriasislichenoides chronica; folliculitis; folliculareczema

Therapy

Isotretinoin; tretinoin; tazarotene

References

Burge S (1999) Management of Darier's disease. Clinical & Experimental Dermatology 24(2):53–56

Darier-White disease

�

Darier disease

Darier’s disease

�

Darier disease

Dark dot disease

�

Reticulate pigmented anomaly

Darling’s disease

�

Histoplasmosis

Day cream for dry skin

�

Alpha hydroxy acids

De Lange syndrome

�

Cornelia de Lange syndrome

De Sanctis-Cacchione syndrome

�

Xeroderma pigmentosum

Decubitus

�

Decubitus ulcer

Decubitus ulcer

Synonym(s)

Decubitus

;

pressure sore

;

pressure ulcer

;

ischemic ulcer

;

bed sore

Definition

Localized area of devitalized tissue second-ary to vascular occlusion from prolongedexternal pressure against an internal bodyprominence, such as the sacrum or heel

Pathogenesis

Microcirculatory occlusion as pressures riseabove capillary filling pressure, resulting inischemia, causing inflammation and tissueanoxia, leading to cell death, tissue necro-sis, and ulceration; paralysis result in mus-cle and soft tissue atrophy, decreasing thebulk over which bony prominences are sup-ported; sensory loss, malnutrition, hypo-


Dental sinus 177

D

proteinemia, and anemia can be contribut-ing factors in prolonged healing time


Stage 1: intact skin with signs of impendingulceration, with blanching erythema fromreactive hyperemiaStage 2: partial-thickness loss of skininvolving epidermis and some dermis;sometimes presenting as an abrasion, blis-ter, or superficial ulcerationStage 3: full-thickness loss of skin withextension into subcutaneous tissue but notthrough the underlying fasciaStage 4: full-thickness loss of skin and sub-cutaneous tissue and extension into mus-cle, bone, tendon, or joint capsule


Pyoderma gangrenosum; squamous cellcarcinoma; factitial ulcer; burn; contactdermatitis; bullous pemphigoid; spider bite;stasis ulcer; vasculitis

Therapy

Reduction or elimination of the source ofexternal pressure, with frequent turning,protective pads, special mattresses, etc

�

;stage 2: hydrocolloid dressings; stages 3 and4: wet dressings; silver sulfadiazine cream;hydrogels; xerogels; daily whirlpool use

References

Walker P (2001) Management of pressure ulcers. Oncology 15(11):1499–1508, 1511

Deep fibromatosis

�

Desmoid tumor

Deer-fly fever

�

Tularemia

Degos’ acanthoma

�


Degos' disease

�

Malignant atrophic papulosis

Degos’ syndrome

�


Delhi boil

�


Dental abscess

�

Oral cutaneous fistula

Dental abscess with sinus tract formation

�


Dental sinus

�



178 Depilatories, chemical

Depilatories, chemical

Trade name(s)

Nair; Neet; Nudit; Magic Shaving Powder;Royal Crown Shaving Powder

Generic available

No

Drug class

Chemical depilatory agent

Mechanism of action

Hydrolysis of hair disulfide bonds

Dosage form

Cream, powder


See table

Common side effects

Cutaneous:

skin irritation


None

Drug interactions

None



References

Ramos-e-Silva M, de Castro MC, Carneiro LV Jr (2001) Hair removal. Clinics in Dermatology 19(4):437–444

Dercum disease

�

Dercum’s disease

Dercum’s disease

Synonym(s)

Dercum’s syndrome

;

Dercum disease

;

adiposis dolorosa

Definition

Disorder in which there are fatty depositsthat apply pressure to the underlyingnerves, resulting in weakness and pain

Pathogenesis

Unknown; autosomal dominant inheritance


Painful, nodular fatty deposits; generalobesity, fatigability, weakness; emotionaldisturbances, such as depression and confu-sion; dementia


Neurofibromatosis; proteus syndrome; pro-gressive lipodystrophy; familial multiplelipomatosis; fibromyalgia; Weber-Christiandisease; multiple symmetrical lipomatosis(Madelung syndrome)

Therapy

Liposuction; surgical excision of painfullipomas

Depilatories, chemical. Dermatologic indications and dosage


Hirsutism Apply as needed Apply as needed

Hypertrichosis Apply as needed Apply as needed

Pseudofolliculitis barbae

Apply as needed Apply as needed


Dermatitis, diaper 179

D

References

Brodovsky S, Westreich M, Leibowitz A, Schwartz Y (1994) Adiposis dolorosa (Dercum's disease): 10-year follow-up. Annals of Plastic Surgery 33(6) 664–668

Dercum’s syndrome

�

Dercum’s disease

Dermal dendrocytoma

�

Dermatofibroma

Dermal duct tumor

�

Poroma

Dermal melanocytoma

�

Blue nevus

Dermatitis artefacta

Synonym(s)

Factitial dermatitis

Definition

Physical or psychological symptoms andsigns intentionally produced or feigned toassume a sick role

PathogenesisExternal trauma, producing skin lesions

Clinical manifestationMultiple, irregularly shaped, eroded orulcerated papules, usually in a distributionwithin easy reach of the dominant hand;blistering sometimes occurring after burns;morphology and distribution not consist-ent with any other dermatosis

Differential diagnosisAtopic dermatitis; scabies; bacterial pyo-derma; herpes simplex virus infection; her-pes zoster; insect bite reaction; polyarteri-tis nodosa; Wegener’s granulomatosis; sep-tic vasculitis; Weber-Christian disease;nodular vasculitis

TherapyUnna boot covering to extremity, ifinvolved; careful evaluation to determine ifsecondary gain something other than psy-chological (monetary, etc); psychiatric con-sultation, if necessary

ReferencesKoblenzer CS (2000) Dermatitis artefacta. Clini-

cal features and approaches to treatment. American Journal of Clinical Dermatology 1(1):47–55

Dermatitis, Berloque


Dermatitis contusiformis

� Erythema nodosum

Dermatitis, diaper

� Diaper dermatitis


180 Dermatitis, exfoliative

Dermatitis, exfoliative

� Exfoliative dermatitis


Synonym(s)Dühring’s disease; Dühring-Bloch disease;hydroa herpetiformis; pemphigus circina-tus

DefinitionImmune-mediated, blistering skin diseasewith an associated gluten-sensitive enterop-athy

PathogenesisGluten main factor in both bowel and skindisease; strong HLA associations (HLA-A1,HLA-B8, HLA DR3, HLA DQw2); unclearpathogenic significance of granular deposi-tion of IgA at the dermal-epidermal junc-tion of the skin

Clinical manifestationTense vesicles on an erythematous base,occurring in tight clusters (herpetiformpattern), symmetrically distributed overextensor surfaces, including elbows, knees,buttocks, shoulders, and the posteriorscalp; occasional occurrence of erosionsand crusts in the absence of vesicles; symp-toms include burning, stinging, and intensepruritus; oral mucosa lesions occur infre-quently; palms and soles usually spared;gastrointestinal symptoms usually mild orabsent

Differential diagnosisBullous pemphigoid; erythema multi-forme; epidermolysis bullosa; epidermoly-sis bullosa acquisita; linear IgA dermatosis;impetigo; pemphigus foliaceus; pemphigusvulgaris; herpes simplex virus infection;herpes zoster

TherapyDapsone�; sulfapyridine 500–1000 mg POtwice daily; gluten-free diet; prednisone

ReferencesReunala TL (2001) Dermatitis herpetiformis.


Dermatitis venenata


Dermatochalasis

Synonym(s)Blepharochalasis; steatoblepharon

DefinitionRedundant and lax eyelid skin and muscle

ReferencesDeAngelis DD, Carter SR, Seiff SR (2002) Derma-

tochalasis. International Ophthalmology Clin-ics 42(2):89–101

Dermatofibroma

Synonym(s)Dermal dendrocytoma; dermatofibromalenticulare; fibroma durum; fibroma sim-plex; histiocytoma; histiocytoma cutis;nodular subepidermal fibrosis; sclerosingangioma; sclerosing hemangioma

DefinitionBenign dermal lesion formed by the prolif-eration of histiocytes or fibroblasts

PathogenesisProbably a reactive tissue change ratherthan a true neoplasm


Dermatofibrosis lenticularis 181

D

Clinical manifestationSolitary, flesh-colored-to-brown, firm,asymptomatic or mildly tender papule;tethering of the overlying epidermis to theunderlying lesion with lateral compression(dimple or button sign); most common onthe extremities; may be multiple lesions

Differential diagnosisNevus; melanoma; seborrheic keratosis;basal cell carcinoma; dermatofibrosarcomaprotuberans; wart; epidermoid cyst; scar;keloid; prurigo nodularis; desmoplastic tri-choepithelioma; foreign body granuloma;mastocytoma; metastasis; juvenile xan-thogranuloma

TherapySurgical excision; shave removal; cryother-apy

ReferencesPariser RJ (1998) Benign neoplasms of the skin.

Medical Clinics of North America 82(6):1285–1307

Dermatofibroma lenticulare

� Dermatofibroma

Dermatofibrosarcoma protuberans

Synonym(s)Bednar tumor; hypertrophic morphea;progressive and recurring dermatofi-broma; fibrosarcoma of the skin

DefinitionLow-grade, locally invasive sarcoma of theskin

PathogenesisCell of origin unclear; possibly fibroblastic,histiocytic, or neuroectodermal

Clinical manifestationBegins as small, asymptomatic papule,most commonly on the trunk or proximalupper extremities; slowly enlarges intoindurated plaque, composed of firm, irreg-ular nodules, varying from flesh-colored toreddish-brown in color

Differential diagnosisDermatofibroma; melanoma; keloid; mor-phea; cutaneous metastasis; lymphoma

TherapyMohs micrographic surgery�; wide, localexcision

ReferencesGloster HM Jr, Harris KR, Roenigk RK (1996) A

comparison between Mohs micrographic sur-gery and wide surgical excision for the treat-ment of dermatofibrosarcoma protuberans. Journal of the American Academy of Dermatol-ogy 35(1):82–87

Dermatofibrosis lenticularis

� Buschke-Ollendorff syndrome

Dermatofibrosarcoma protuberans. Indurated plaque with irregular nodules


182 Dermatofibrosis lenticularis disseminata with osteopoikilosis

Dermatofibrosis lenticularis disseminata with osteopoikilosis


Dermatographism

Synonym(s)Dermographism; factitious urticaria; skinwriting

DefinitionLinear, urticarial wheal which occurs withinminutes of vigorously stroking the skinwith an object

PathogenesisUnclear; skin trauma possibly releases anantigen that interacts with the membrane-bound immunoglobulin E (IgE) of mastcells, releasing inflammatory mediators,particularly histamine, resulting leakage insmall blood vessels; no association withsystemic diseases, food allergies, oringested medications

Clinical manifestationUrticarial wheals develop within 5 minutesof stroking the skin and persist for 15–30 minutes; resolve without residua

Differential diagnosisChronic urticaria; contact urticaria; insectbite reaction; mastocytosis

TherapyAntihistamines, second generation�

ReferencesLee EE, Maibach HI (2001) Treatment of urticaria.

An evidence-based evaluation of antihista-mines. American Journal of Clinical Dermatol-ogy 2(1):27–32

Dermatoheliosis

� Actinic elastosis

Dermatomegaly

� Cutis laxa

Dermatomycosis furfuracea


Dermatomycosis nigricans

� Tinea nigra

Dermatomyofibroma

Synonym(s)Plaque-like dermal fibromatosis

DefinitionBenign dermal proliferation consisting offibroblasts and myofibroblasts

PathogenesisUnknown

Clinical manifestationSolitary, asymptomatic, slow growing,flesh-colored-to-red, firm plaque, occur-ring in women, often around the axilla

Differential diagnosisMorphea; lichen sclerosus; dermatofi-broma; scar; keloid; dermatofibrosarcomaprotuberans; desmoid; leiomyoma; myofi-


Dermatosis papulosa nigra 183

D

broma; neurofibroma; granuloma annu-lare; sarcoidosis


ReferencesRose C, Brocker EB (1999) Dermatomyofibroma:

case report and review. Pediatric Dermatology 16(6):456–459

Dermatomyositis

Synonym(s)Idiopathic inflammatory myopathy;dermatomyositis sine myositis; amyopathicdermatomyositis

DefinitionInflammatory myopathy with characteris-tic cutaneous findings

PathogenesisPossible etiologic factors: genetic predispo-sition; immunologic abnormalities; infec-tions; concomitant medication use

Clinical manifestationSkin disease sometimes initial or sole mani-festation; muscle disease occurring concur-rently, sometimes preceding skin disease orfollowing skin disease by weeks to years;eruption photodistributed and photo-exac-erbated; violaceous-to-dusky, erythema-tous plaques with or without edema in asymmetrical distribution involving perior-bital skin; central facial erythemaScalp involvement: erythematous to viola-ceous, psoriasiform plaques; slightly ele-vated, violaceous papules and plaques;Gottron papules over bony prominences,particularly the metacarpophalangealjoints, the proximal interphalangeal joints,and/or the distal interphalangeal jointsSimilar lesions overly the elbows, knees,and/or feet; periungual telangiectases;irregular, ragged cuticles with hypertrophyand hemorrhagic infarcts; calcinosis of the

skin or muscle common in children or ado-lescentsMuscle disease: proximal symmetrical mus-cle weakness; associated with internalmalignancies in patients older than 50 yearsold

Differential diagnosisLupus erythematosus; psoriasis; lichen pla-nus; scleroderma; seborrheic dermatitis;pemphigus foliaceus; polymorphous lighteruption; dermatophytosis; parapsoriasis;rosacea; sarcoidosis

TherapyPrednisone�; steroid-sparing drugs –methotrexate; azathioprine; cyclophospha-mide; cyclosporine, mycophenolate mofetil;hydroxychloroquine; methotrexate; IVIG1 gm IV on 2 successive days, repeated every4–6 weeks as needed; calcinosis cutis: surgi-cal excision of symptomatic lesions

ReferencesOlsen NJ, Park JH, King LE Jr (2001) Amyopathic

dermatomyositis. Current Rheumatology Re-ports 3(4):346–351

Dermatomyositis sine myositis

� Dermatomyositis

Dermatosis cenicienta

� Ashy dermatosis

Dermatosis papulosa nigra

Synonym(s)None


184 Dermite pigmentée en forme de coulée

DefinitionSkin condition characterized by multiple,small, hyperpigmented papules on the faceof adult blacks

PathogenesisProbably genetically determined; hamar-tomatous developmental defect of thepilosebaceous follicle

Clinical manifestationMultiple, firm, smooth, dark-brown-to-black, flattened papules, mainly on themalar area of the face and the forehead; firstappear after puberty; new lesions occurthroughout life

Differential diagnosisWart; nevus; acrochordon; adenomasebaceum; seborrheic keratosis

TherapyLight electrodesiccation and curettage; cry-otherapy

ReferencesKauh YC, McDonald JW, Rapaport JA, Ruschak PJ,

Luscombe HA (1983) A surgical approach for dermatosis papulosa nigra. International Jour-nal of Dermatology 22(10):590–592

Dermite pigmentée en forme de coulée


Dermographism

� Dermatographism

Dermoid

� Dermoid cyst

Dermoid cyst

Synonym(s)Choristoma; dermoid; lipodermoid

DefinitionSubcutaneous cysts of ectodermal origin,arising along embryonic fusion planes

PathogenesisSequestrations of cutaneous epithilium dur-ing fetal development

Clinical manifestationOccur most commonly on the head andneck, particularly over the supraorbitalregion, glabella, upper eyelid and scalp;appear as subcutaneous masses, sometimeswith a dimple or sinus tract; with deeperextension, lesion feel bound to underlyingperiosteum; sometimes contain nails, den-tal structures, cartilage-like and bone-likematerial, and fat

Differential diagnosisEpidermoid cyst; pilomatricoma; metasta-sis; meningocele; encephalocele; nevussebaceous; thyroglossal duct cyst; cutane-ous ectopic brain; lymph node


ReferencesOgle RF, Jauniaux E (1999) Fetal scalp cysts–di-

lemmas in diagnosis. Prenatal Diagnosis 19(12):1157–1159


Dexamethasone 185

D

Dermolytic pemphigoid

� Epidermolysis bullosa acquisita

Desert rheumatism

� Coccidioidomycosis

Desmoid

� Desmoid tumor

Desmoid tumor

Synonym(s)Desmoid; musculoaponeurotic fibromato-sis; aggressive fibromatosis; deep fibroma-tosis; non-metastasizing fibrosarcoma

DefinitionBenign fibrous neoplasm, related tofibromatosis, originating from the muscu-loaponeurotic structures, usually on theabdominal wall

PathogenesisUncertain; possibly related to genetic fac-tors, trauma, or hormonal factors; myofi-broblast is the cell responsible for tumorgrowth

Clinical manifestationSolitary, slow-growing, firm, smooth,mobile mass, most commonly in the ante-rior abdominal wall and shoulder girdle;history of trauma (often surgical) to the siteof tumor development; often adherent tosurrounding structures; locally invasive, butnot metastatic; overlying skin usually unaf-fected

Differential diagnosisDermatofibrosarcoma protuberans; metas-tasis; leiomyosarcoma; Gardner syndrome

TherapyWide surgical resection�; radiation therapy

ReferencesShields CJ, Winter DC, Kirwan WO, Redmond HP

(2001) Desmoid tumours. European Journal of Surgical Oncology 27(8):701–706

Desonide

� Corticosteroids, topical, low potency

Desoximetasone


Desquamative gingivitis

DefinitionInflammation of the outermost soft tissueof the gums, which become red, formsuperficial erosions, lose their normalshape, and bleed easily; most often seen inpatients with cicatricial pemphigoid

ReferencesFleming TE, Korman NJ (2000) Cicatricial pem-

phigoid. Journal of the American Academy of Dermatology 43(4):571–591

Dexamethasone



186 Diabetic bulla

Diabetic bulla


Diabetic bullae


Diabetic dermopathy

Synonym(s)Shin spots; pigmented pretibial patches;diabetic microangiopathy; spotted leg syn-drome

DefinitionHyperpigmented, atrophic lesions on thelegs of patients with diabetes mellitus

PathogenesisUncertain; possibly microangiopathy;trauma with poor wound healing

Clinical manifestationSmall, brown, atrophic papules on the ante-rior legs, appearing singly or in groups

Differential diagnosisLupus erythematosus; lichen planus; post-traumatic scars; benign pigmented pur-pura; lichen sclerosus; morphea

TherapyNone

ReferencesRomano G, Moretti G, Di Benedetto A, Giofre C,

Di Cesare E, Russo G, Califano L, Cucinotta D (1998) Skin lesions in diabetes mellitus: preva-lence and clinical correlations. Diabetes Re-search & Clinical Practice – Supplement 39(2):101–106

Diabetic microangiopathy

� Diabetic dermopathy

Diaper dermatitis

Synonym(s)Diaper rash, perianal dermatitis

DefinitionIrritant contact dermatitis caused by over-hydration of the skin, maceration, pro-longed contact with urine and feces,retained diaper soaps, and irritating topi-cal preparations

PathogenesisIncreased wetness makes the skin more sus-ceptible to damage by physical, chemical,and enzymatic mechanisms; urease enzymefound in the stratum corneum liberatesammonia from cutaneous bacteria; lipasesand proteases in feces mix with urine oneroded skin, and cause an alkaline surfacepH; bile salts in the stools enhance activityof fecal enzymes; Candida albicans possi-ble cause or effect of eruption; childrenwith history of atopic dermatitis possiblymore susceptible

Clinical manifestationErythematous scaly diaper area, often withfissures and erosions; sometimes patchy orconfluent; affects the abdomen from theumbilicus to the thighs, encompassing thegenitalia, perineum, and buttocks; geni-tocrural folds spared

Differential diagnosisPsoriasis; atopic dermatitis; allergic contactdermatitis; biotin deficiency; acrodermati-tis enteropathica; candidiasis; scabies;Langerhans cell histiocytosis; child abuse


Dicloxacillin 187

D

TherapyChanging of diapers frequently and/or leav-ing inflamed area uncovered for as long aspossible between diaper changes�; zincoxide paste; white petrolatum

ReferencesWolf R, Wolf D, Tuzun B, Tuzun Y (2000) Diaper

dermatitis. Clinics in Dermatology 18(6):657–660

Diaper rash


Dicloxacillin

Trade name(s)Dynapen


Drug classPenicillin antibiotic

Mechanism of actionInhibition of penicillin-binding proteinscauses blockade of bacterial cell wall syn-thesis



Common side effectsCutaneous: urticaria and other skin erup-tionsGastrointestinal: nausea, vomiting,diarrhea

Serious side effectsBone marrow: thrombocytopeniaCutaneous: anaphylaxis, Stevens-Johnsonsyndrome, toxic epidermal necrolysisGastrointestinal: pseudomembranous coli-tisRenal: interstitial nephritis

Drug interactionsAminoglycosides; oral contraceptives;methotrexate; probenecid

Contraindications/precautionsHypersensitivity to drug class or compo-nent; use with caution in patients with

Dicloxacillin. Dermatologic indications and dosage


Cellulitis 250–500 mg PO 4 times daily for 7–10 days

25–50 mg per kg PO 4 times daily for 7–10 days

Ecthyma 250–500 mg PO 4 times daily for 7–10 days


Erysipelas 250–500 mg PO 4 times daily for 7–10 days


Impetigo 250–500 mg PO 4 times daily for 7–10 days


Staphylococcal scalded skin syndrome

250–500 mg PO 4 times daily for 7–10 days

< 40 kg – 12.5 mg per kg daily PO divided into 4 doses; > 40 kg – 125 mg PO 4 times daily


188 Diffuse nonepidermolytic palmoplantar keratoderma

cephalosporin allergy, seizure disorder,impaired renal function

ReferencesSalkind AR, Cuddy PG Foxworth JW (2001) The

rational clinical examination. Is this patient al-lergic to penicillin? An evidence-based analysis of the likelihood of penicillin allergy. Journal of the American Medical Association 285(19):2498–2950

Diffuse nonepidermolytic palmoplantar keratoderma


Diffuse systemic sclerosis

� Progressive systemic sclerosus

Diflorasone diacetate

� Corticosteroids, topical, super potency

Digital duplication

� Supernumerary digit

Digital fibrokeratoma, acquired

� Acquired digital fibrokeratoma

Digital fibrous tumor of childhood

� Infantile digital fibromatosis

Digital mucinous pseudocyst


Digital mucoid cyst


Digital mucous cyst

Synonym(s)Cystomata; myxomatous cutaneous cyst;myxomatous degenerative cyst; mucouscyst; myxoid cyst; synovial cyst; digitalmucoid cyst; digital myxoid cyst; digitalmucinous pseudocyst

DefinitionSoft, cystic papule of the digits, containingmucinous material

PathogenesisArises from mucoid degeneration of con-nective tissue; osteophytes in those withosteoarthritis possibly a stimulus

Clinical manifestationSolitary, round-to-oval, dome-shaped,papule, with normal overlying skin; con-tains a viscous, gelatinous, clear or yellow-tinged fluid


Dilated pore of Winer 189

D

Differential diagnosisEpidermoid cyst; fibrokeratoma; giant-celltendon sheath tumor; Heberden node;myxoid malignant fibrous histiocytoma;myxoid variant of liposarcoma; rheumatoidnodule; gouty tophus; subcutaneous granu-loma annulare

TherapyIntralesional triamcinolone 3–5 mg per ml;cryotherapy; incision and drainage;destruction by electrodesiccation; surgicalexcision

Referencesde Berker D, Goettman S, Baran R (2002) Subun-

gual myxoid cysts: clinical manifestations and response to therapy. Journal of the American Academy of Dermatology 46(3):394–398

Digital myxoid cyst


Digital papillary adenoma

� Aggressive digital papillary ade-noma

Digitate dermatitis


Digitate dermatosis


Dilated pore

Synonym(s)Winer’s pore; Winer’s dilated pore; dilatedpore of Winer; giant follicle; enlarged soli-tary comedone

DefinitionHair structure anomaly appearing as anenlarged, solitary comedone

PathogenesisUnknown; neoplasm of the intraepidermalfollicle and infundibulum of pilosebaceousapparatus

Clinical manifestationSolitary large comedone on the face ortrunk, most commonly the back; lateralpressure yields keratinous material

Differential diagnosisEpidermoid cyst; trichoepithelioma; solarcomedone; pilar sheath acanthoma; seba-ceous trichofolliculoma

TherapyExpression of comedone contents, followedby electrodesiccation of the base; surgicalexcision

ReferencesToshitani A; Imayama S, Urabe A, Kiryu H, Hori Y

(1996) Hair cortex comedo. American Journal of Dermatopathology 18(3):322–325

Dilated pore of Winer

� Dilated pore


190 Diphtheria, cutaneous

Diphtheria, cutaneous

Synonym(s)None

DefinitionAcute, toxin-mediated disease caused byCorynebacterium diphtheriae

PathogenesisC. diphtheriae (causative organism) an aer-obic, toxin-producing, gram-positive bacil-lus; toxin production only when the bacil-lus infected by a specific virus carrying thegenetic information for the toxin; only toxi-genic strains cause severe disease; toxininhibiting cellular protein synthesis respon-sible for local tissue destruction and mem-brane formation; toxin produced at the siteof the membrane absorbed into the blood-stream and disseminated

Clinical manifestationSkin findings: seen mainly in homeless per-sons; erythematous scaling plaques; ulcerswith an overlying membrane and demar-cated edges; mucous membranes some-times involved

Differential diagnosisOther bacterial pyodermas; erythema mul-tiforme; tropical ulcer; pyoderma gan-grenosum; Majocchi’s granuloma; atypicalmycobacterial infection; nocardiosis;aspergillosis; syphilis; granuloma inguinale;chancroid

TherapyErythromycin; procaine penicillin G; diph-theria antitoxin 20,000–50,000 units IM�

ReferencesEfstratiou A, Roure C (2000) The European Labo-

ratory Working Group on diphtheria: A global microbiologic network. Journal of Infectious Diseases 181 Suppl 1:S146–151

Discoid eczema

� Nummular eczema

Discoid lupus erythematosus

� Lupus erythematosus, discoid

Discrete keratoderma

� Knuckle pads

Dissecting cellulitis

� Dissecting cellulitis of scalp

Dissecting cellulitis of scalp

Synonym(s)Dissecting cellulitis; perifolliculitis capitisabscedens et suffodiens; Hoffman’s disease

DefinitionChronic inflammatory disease character-ized by painful suppurating lesions of thescalp, leading to scarring alopecia

PathogenesisAssociated with acne conglobata, hidraden-itis suppurativa, and pilonidal cysts, all ofwhich have follicular blockage as the com-mon mechanism; retained material dilatesand causes follicular rupture; keratin andorganisms from the damaged hair folliclesinitiate neutrophilic and granulomatousresponse; bacterial infection secondaryevent


Dove aerosol 191

D

Clinical manifestationPerifollicular pustules; tender nodules(some discharging pus or gelatinous mate-rial); intercommunicating sinuses betweennodules; patchy alopecia with scarring ; fre-quent recurrences over many years

Differential diagnosisFolliculitis keloidalis; folliculitis decalvans;kerion; pseudopelade of Brocq; lichen plan-opilaris; bacterial pyoderma

TherapyIsotretinoin; dapsone; intralesional triamci-nolone 5 mg per ml; laser hair removal;wide local excision

ReferencesSullivan JR, Kossard S (1999) Acquired scalp alo-

pecia. Part II: A review. Australasian Journal of Dermatology 40(2):61–70

Disseminated cat-scratch disease


Disseminated gonococcal infection

� Gonococcemia

Disseminated lenticular dermatofibrosis


Disseminated superficial actinic porokeratosis

� Porokeratosis

Donohue syndrome

� Leprechaunism

Donovanosis

� Granuloma inguinale

Dorfman Chanarin syndrome


Double lip and nontoxic thyroid enlargement syndrome


Dove aerosol



192 Dowling Degos Ossipowski disease

Dowling Degos Ossipowski disease

� Reticulate pigmented anomaly

Dowling-Degos disease

� Reticulate pigmented anomaly

Doxepin


Doxycycline

Trade name(s)Vibramycin; Doryx; Vibra-Tabs; Monodox


Drug classTetracycline

Mechanism of actionAntibiotic activity: protein synthesis inhibi-tion by binding to the 30S ribosomal subu-nit; anti-inflammatory activity: unclearmechanisms

Dosage form50 mg, 100 mg tablets


Common side effectsCutaneous: photosensitivity, stomatitis,oral candidiasis, urticaria or other vascularreaction

Gastrointestinal: nausea and vomiting,diarrhea, esophagitisNeurologic: tinnitus, dizziness, drowsiness,headache, ataxia

Serious side effectsGastrointestinal: pseudomembranous coli-tis, hepatotoxicityHematologic: neutropenia, thrombocytope-niaNeurologic: pseudotumor cerebri

Drug interactionsAntacids; calcium salts; oral contraceptives;digoxin; iron salts; isotretinoin; magne-sium salts; warfarin

Contraindications/precautionsHypersensitivity to drug class or compo-nent; pregnancy; patient < 8 years old; cau-tion if impaired renal or liver function



Drug-induced bullous photosensitivity

� Pseudoporphyria

Dry skin

� Asteatosis� Xerosis

Dryness of skin

� Xerosis


Dryness of skin 193

D

Doxycycline. Dermatologic indications and dosage


Acne vulgaris 50–100 mg PO twice daily > 8 years old – 50–100 mg PO twice daily

Anthrax 100 mg PO twice daily for 60 days in bioterrorism situation

> 8 years old – 50 mg PO twice daily for 60 days in bioterrorism situation

Atrophoderma of Pasini-Pierini

50–100 mg PO twice daily > 8 years old – 50 mg PO twice daily

Bacillary angiomatosis 100 mg PO twice daily for 3 weeks > 8 years old – 100 mg PO twice daily for 3 weeks

Bartonellosis 100 mg PO twice daily for 3 weeks > 8 years old – 50 mg PO twice daily for 3 weeks

Boutonneuse fever 200 mg PO or IV immediately and at bedtime, followed by 100 mg PO twice daily for 3 days

> 8 years old – 2–5 mg per kg PO daily for 7–10 days

Bullous pemphigoid 50–100 mg PO twice daily > 8 years old – 50 mg PO twice daily



Epidemic typhus 200 mg PO or IV twice daily for 3 days, then 100 mg PO of IV daily until 48–72 hours after patient becomes afebrile

> 8 years old – 200 mg PO or IV twice daily for 3 days, then maintenance dose 100 mg PO or IV twice daily until 48–72 hours after patient becomes afebrile

Folliculitis 50–100 mg PO twice daily > 8 years old – 50 mg PO twice daily

Leptospirosis 100 mg PO twice daily for 3 weeks > 8 years old – 50 mg PO twice daily for 3 weeks



Lyme disease 100 mg PO twice daily for 21 days; prophylaxis after tick bite – 200 mg PO for 1 dose

> 8 years old – 50 mg PO twice daily for 3 weeks

Lymphogranuloma venereum

100 mg PO twice daily for 3 weeks > 8 years old – 50 mg PO twice daily for 3 weeks

Perioral dermatitis 100 mg PO twice daily for at least 30 days

> 8 years old – 50–100 mg PO twice daily for at least 30 days

Relapsing fever 100 mg PO twice daily for 7 days > 8 years old – 50 mg PO twice daily for 7 days

Rickettsialpox 100 mg PO twice daily for 5 days > 8 years old – 50 mg PO twice daily for 5 days

Rocky Mountain spotted fever

100 mg PO twice daily for 7–10 days > 8 years old – 2 mg per kg PO or IV loading dose, followed by 1 mg per kg PO or IV every 12 hours for 7 days and for at least 48 hours after defervescence

Rosacea 100 mg PO twice daily for at least 30 days



194 Drysol

Drysol


DSAP

� Porokeratosis

Dühring-Bloch disease

� Dermatitis herpetiformis

Dühring’s disease


Dupuy's syndrome

� Auriculotemporal syndrome

Dupuytren’s contracture

Synonym(s)Palmoplantar fibromatosis; Dupuytren'sdisease, palmar fasciitis; Viking disease

DefinitionDisorder characterized by subcutaneousfascia thickening and shortening, causingthe fingers to retract down towards thepalm of the hand

PathogenesisUnclear; dominant genetic inheritance;often involves individuals of northern Euro-pean descent; trauma sometimes initiatesor accelerates the process; associated withalcoholism, diabetes mellitus, smoking, epi-lepsy, pulmonary disease

Clinical manifestationAsymptomatic, palmar skin nodule, gener-ally within the distal aspect of the palm,often with puckering of the skin above thenodularity; overlying skin sometimesadherent to the fascia, and fibrous cordsometimes extending into the finger; ringfinger most commonly involved site, fol-lowed by the small finger

Differential diagnosisTrigger finger

Scrub typhus 100 mg PO twice daily for 7–14 days > 8 years old – 50 mg PO twice daily for 14 days

Trench fever 100 mg PO twice daily for 4 weeks > 8 years old – 50 mg PO twice daily for 4 weeks

Tularemia 100 mg PO twice daily for 7–14 days or until patient is afebrile for 5–7 days

> 8 years old – 50 mg PO twice daily for 7–14 days or until patient is afebrile for 5–7 days

Yaws 100 mg PO twice daily for 15 days > 8 years old – 2–5 mg per kg PO divided into 2 doses daily for 15 days

Doxycycline. Dermatologic indications and dosage (Continued)



Dyshidrotic eczema 195

D

TherapyPhysical therapy in early stages�; intrale-sional triamcinolone 3–5 mg per ml; partialsurgical fasciectomy for a patient with sig-nificant functional disability

ReferencesSaar JD, Grothaus PC (2000) Dupuytren's disease:

An overview. Plastic & Reconstructive Surgery 106(1):125–134

Dupuytren's disease

� Dupuytren’s contracture

Dwarfism with retinal atrophy and deafness

� Cockayne syndrome

Dyschondrodysplasia with hemangiomas

� Maffucci syndrome

Dyshidrosis

� Dyshidrotic eczema

Dyshidrotic eczema

Synonym(s)Dyshidrosis; pompholyx; vesicular pal-moplantar eczema; vesicular eczema ofpalms and soles

DefinitionRecurrent or chronic relapsing form ofvesicular palmoplantar dermatitis

PathogenesisOccurring commonly in atopic individuals;associated with stress, infection, exogenouscontactants, climate changes

Clinical manifestationSymmetric crops of clear vesicles and/orbullae on the palms and lateral aspects offingers and feet; vesicles deep seated, with atapioca-like appearance, and sometimesbecoming confluent to form bullae; maydevelop crusting, scaling, and fissuringafter persistent scratching

Differential diagnosisContact dermatitis; vesicular tinea pedis;tinea manus; palmoplantar pustular psoria-sis; autosensitization reaction (id reaction)

TherapyCorticosteroid, topical, high potency�;severe flare: prednisone; triamcinolone 40–80 mg IM as single dose.Chronic persistent disease: azathioprine;local photochemotherapy; disulfiram 250–500 mg PO per day in nickel-sensitivepatients; aluminium acetate 5% solutionsoaks

ReferencesLandow K (1998) Hand dermatitis. The perennial

scourge. Postgraduate Medicine 103(1):141–142, 145–148, 151–152

Dyshidrotic eczema. Multiple vesicles on the hands, with concentration along the sides of the digits


196 Dyskeratoma, warty

Dyskeratoma, warty

� Warty dyskeratoma

Dyskeratosis congenita

Synonym(s)Zinsser-Engman-Cole syndrome; Zinsser-Cole-Engman syndrome

DefinitionGenodermatosis characterized by reticu-lated hyperpigmentation, nail dystrophy,premalignant leukoplakia of the oralmucosa, and progressive pancytopenia

PathogenesisMutations in DKC1 cause X-linked recessiveform; involved in the regulation of the pro-liferative capacity of the cell; defect inmaintenance of telomeres results in chro-mosomal instability, telomeric rearrange-ments, and cancer progression; etiology ofautosomal dominant and autosomal reces-sive forms unknown

Clinical manifestationCutaneous manifestations developingbetween 5 and 15 years of age; tan-to-gray,hyperpigmented or hypopigmented mac-ules and patches in a mottled, or reticu-lated pattern, sometimes with poikilo-derma; located on the upper trunk, neck,and face, often with involvement of sun-exposed areas; scalp alopecia; mucosal leu-koplakia on the buccal mucosa, tongue,oropharynx, esophagus, urethral meatus,glans penis, lacrimal duct, conjunctiva,vagina, anus; dental caries; progressive naildystrophy; increased incidence of malig-nant neoplasms, particularly squamous cellcarcinoma of the skin, mouth, nasophar-ynx, esophagus, rectum, vagina, and cer-

vix; late bone marrow failure; pulmonarycomplications

Differential diagnosisGraft versus host disease; Fanconi syn-drome; Rothmund-Thompson syndrome;ataxia telangiectasia

TherapyNo therapy for skin disease; bone marrowtransplantation

ReferencesDokal I (2000) Dyskeratosis congenita in all its

forms. British Journal of Haematology 110(4):768–779

Dysplasia epiphysialis punctata

� Conradi disease

Dysplastic mole

� Atypical mole

Dysplastic nevus

� Atypical mole

Dystrophic epidermolysis bullosa

� Epidermolysis bullosa


E

Early-onset prurigo of pregnancy

�

Prurigo of pregnancy

Eccrine acrospiroma

Synonym(s)

Acrospiroma

;

myoepithelioma

;

clear cellhidradenoma

;

clear cell adenoma

;

cystichidradenoma

;

sweat gland adenoma

;

eccrine sweat gland adenoma

Definition

Tumor of eccrine sweat gland origin, with apredominance of clear cells

Pathogenesis

Unknown


Onset after minor trauma; solitary, flesh-colored dermal papule; occurring mostcommonly on the scalp, face, and trunk;tendency for central ulceration; occasionalmalignant degeneration


Basal cell carcinoma; lymphangioma;hemangioma; squamous cell carcinoma

Therapy

Surgical excision

�

References

Ishikawa M, Nakanishi Y, Yamazaki N, Yamamoto A (2001) Malignant eccrine spiradenoma: A case report and review of the literature. Derma-tologic Surgery 27(1):67–70

Eccrine adenocarcinoma

�

Eccrine carcinoma

Eccrine bromhidrosis

�

Bromhidrosis

Eccrine carcinoma

Synonym(s)

Eccrine adenocarcinoma

;

malignant tumorwith eccrine differentiation

Definition

Neoplasm of eccrine sweat gland withpotential for destructive local tissue infil-tration and metastasis; sometimes subdi-vided into tumors arising de novo in nor-mal skin and tumors originating from pre-existing, benign, sweat gland tumors


198 Eccrine chromhidrosis

Pathogenesis

Derived from any portion of the eccrineapparatus or resulting from the malignanttransformation of an existing benigneccrine tumor


Non-specific solitary nodule or plaque withoccasional ulceration, on the head, extremi-ties, or trunk


Basal cell carcinoma; squamous cell carci-noma; Merkel cell carcinoma; cutaneousmetastasis; eccrine acrospiroma; micro-cystic adnexal carcinoma; eccrine porocar-cinoma; cutaneous adenoid cystic carci-noma

Therapy

Wide local excision; Mohs micrographicsurgery; radiation therapy

References

Katzman BM, Caligiuri DA, Klein DM, DiMaio TM, Gorup JM (1997) Eccrine carcinoma of the hand: a case report. Journal of Hand Surgery – American Volume 22(4):737–739

Eccrine chromhidrosis

�

Chromhidrosis

Eccrine cystadenoma

�

Eccrine hidrocystoma

Eccrine hidradenoma

Synonym(s)

Clear cell hidradenoma

;

clear cell myoepi-thelioma

;

solid cystic hidradenoma

Definition

Skin tumor of sweat gland origin with dis-tinctive histologic appearance

Pathogenesis

Unknown


Solitary, dome-shaped papule or nodule,often attached to the overlying epidermis;associated epidermal thickening or ulcera-tion; most common over scalp, face, andtrunk


Basal cell carcinoma; squamous cell carci-noma; dermatofibroma; epidermoid cyst

Therapy

Surgical excision

�

References

Hernández-Perez E, Cestoni-Parducci R (1985) Nodular hidradenoma and hidradenocarcino-ma. Journal of the American Academy of Der-matology 12:15–20


Synonym(s)

Eccrine cystadenoma

;

eccrine syringocysta-denoma

;

syringectasia

Definition

Tumor consisting of a cystic proliferation ofeccrine secretory elements

Pathogenesis

Possibly adenomatous cystic proliferationsof the eccrine glands or retention cysts ofthe eccrine sweat apparatus


Asymptomatic, solitary, translucent-to-blu-ish papule, with a predilection for the peri-orbital area


Ecthyma 199

E


Apocrine hidrocystoma; basal cell carci-noma; epidermoid cyst; mucous cyst; syrin-goma; milium; steatocystoma multiplex

Therapy

Incision and drainage, followed by surgicaldestruction of the cyst wall by light electro-desiccation and curettage; punch, shave, orelliptical excision

References

Alfadley A, Al Aboud K, Tulba A, Mourad MM (2001) Multiple eccrine hidrocystomas of the face. International Journal of Dermatology 40(2):125–129

Eccrine poroma

�

Poroma

Eccrine spiradenoma

�

Spiradenoma

Eccrine sweat gland adenoma

�

Eccrine acrospiroma

Eccrine syringocystadenoma

�


Echovirus 16 infection

�

Boston exanthem

Econazole

�

Azole antifungal agents

Ecthyma

Synonym(s)

Pyoderma

Definition

Skin infection that invades into the dermis,most often caused by organism Streptococ-cus

Pathogenesis

Caused by bacterial infection, usually Strep-tococcus but sometimes Staphylococcus;predisposing factors: previous tissue injury,immunocompromised state; environmentalfactors: high temperature and humidity,crowded living conditions, poor hygiene


Begins as a vesicle or pustule, ulceratingand producing a yellowish crust with ery-thematous, indurated borders


Herpes simplex virus infection; atypicalmycobacterial infection; nocardia infec-tion; sporotrichosis; trauma; insect or spi-der bite reaction; pyoderma gangrenosum

Therapy

Mupirocin ointment applied 3 times dailyfor 7–10 days; dicloxacillin; cephalexin;known Streptococcal infection: penicillin

�


200 Ecthyma contagiosum

References

Mancini AJ (2000) Bacterial skin infections in children: the common and the not so common. Pediatric Annals 29(1):26–35

Ecthyma contagiosum

�

Orf

Ecthyma gangrenosum

Synonym(s)

None

Definition

Cutaneous manifestation of Pseudomonasaeruginosa bacteremia, usually occurringin patients who are critically ill and/orimmunocompromised

Pathogenesis

Caused by Pseudomonas aeruginosa, agram negative bacterial pathogen whichdisseminates in patients with impaired cel-lular or humoral immunity or those withsevere underlying illnesses such as severeburns, malnutrition, recent chemotherapy,immunosuppressive therapy, or diabetesmellitus


Appears as edematous, well-circumscribedplaques, rapidly evolving into hemorrhagicbullae, spreading peripherally, and eventu-ally turning into a black necrotic ulcer withan erythematous rim; commonly occurs inthe gluteal or perineal region or extremi-ties; sign of widespread dissemination ofinfection


Ecthyma; herpes simplex virus infection;atypical tuberculosis; nocardiosis; sporotri-

chosis; trauma; gram negative folliculitis;pyoderma gangrenosum; septicemia fromother infectious agents; cryoglobulinemia;polyarteritis nodosa; necrotizing fasciitis;vasculitis

Therapy

Initial therapy: antipseudomonal penicillin(piperacillin) with an aminoglycoside (gen-tamicin).Subsequent therapy based on culture sensi-tivity

References

Khan MO, Montecalvo MA, Davis I, Wormser GP (2000) Ecthyma gangrenosum in patients with acquired immunodeficiency syndrome. Cutis 66(2):121–123

Ecthyma infectiosum

�

Orf

Ectodermal dysplasia absent dermatoglyphics

�

Basan syndrome

Ectodermal dysplasia, anhidrotic

�


Ectodermal dysplasia, hidrotic

�

Hidrotic ectodermal dysplasia


Eczematidlike purpura of Doucas and Kapetanakis 201

E

Ectodermal dysplasia, hypohidrotic

�


Eczema craquelatum

�

Asteatotic eczema

Eczema craquelé

�

Asteatotic eczema

Eczema fendille

�

Asteatotic eczema

Eczema herpeticum

Synonym(s)

Kaposi varicelliform eruption

;

eczema vac-cinatum

Definition

Eruption caused by herpes simplex virus(HSV)-1, herpes simplex virus (HSV)-2,Coxsackie A16 virus, or vaccinia virus thatinfects a preexisting dermatosis, most com-monly atopic dermatitis

Pathogenesis

Caused by herpes simplex virus (HSV)-1,herpes simplex virus (HSV)-2, CoxsackieA16 virus, or vaccinia virus infecting a pre-existing dermatosis; possibly associatedwith local T-cell immune defect, low NKcells, and/or a low antibody titer against theinfective organism


Presents as clusters of umbilicated vesiculo-pustules in areas where the skin has beenaffected by a preexistent dermatitis; umbili-cated vesiculopustules progress to ero-sions, usually over the upper trunk andhead; vesicles often become hemorrhagicand crusted, coalescing to form large,denuded plaques that bleed and sometimesbecome secondarily infected with bacteria


Impetigo; varicella; contact dermatitis; bul-lous pemphigoid; dermatitis herpetiformis;erythema multiforme; pemphigus

Therapy

Acyclovir; valacyclovir

References

Mooney MA, Janniger CK, Schwartz RA (1994) Kaposi's varicelliform eruption. Cutis 53(5):243–245

Eczema hiemalis

�

Asteatotic eczema

Eczema marginatum

�

Tinea cruris

Eczema vaccinatum

�

Eczema herpeticum

Eczematidlike purpura of Doucas and Kapetanakis

�

Benign pigmented purpura


202 Eczematoid epitheliomatous dermatosis

Eczematoid epitheliomatous dermatosis

�

Paget’s disease

Effluvium, anagen

�

Anagen effluvium

Effluvium, telogen

�

Telogen effluvium

Eflornithine

Trade name(s)

Vaniqa

Generic available

No

Drug class

Ornithine decarboxylase inhibitor

Mechanism of action

Possibly related to ornithine decarboxylaseinhibition, which decreases hair growth

Dosage form

13.9% cream


See table

Common side effectsCutaneous: stinging; burning sensation,irritant contact dermatitis, acneform erup-tion, pseudofolliculitis barbae




ReferencesHickman JG, Huber F, Palmisano M (2001) Hu-

man dermal safety studies with eflornithine HCl 13.9% cream (Vaniqa), a novel treatment for excessive facial hair. Current Medical Re-search & Opinion 16(4):235–244

Ehlers Danlos syndrome

Synonym(s)Cutis hyperelastica

DefinitionHeterogeneous group of inherited connec-tive tissue disorders characterized by jointhypermobility, skin fragility, and hyperex-tensibility

PathogenesisSpecific collagen defect has been identifiedin 6 of the 11 types: Type IV – decreased

Eflornithine. Dermatologic indications and dosage


Hypertrichosis Apply twice daily Apply twice daily


Elastolysis 203

E

type III collagen; types V and VI – deficien-cies in hydroxylase and lysyl oxidase; typeVII – amino-terminal procollagen pepti-dase deficiency; type IX – abnormal coppermetabolism; type X – nonfunctioningplasma fibronectin

Clinical manifestationFindings common to all subtypes: skinhyperextensible, doughy, white, and soft,with underlying vessels sometimes visible;small, spongy tumors (molluscoid pseudo-tumors) over scars and pressure points;smaller palpable, and movable calcifiednodules in subcutaneous tissue; nodules inarms and over tibias; skin fragility, with fre-quent bruises, lacerations, and poor woundhealing; hyperextensible joints, with fre-quent dislocations

Differential diagnosisPseudoxanthoma elasticum; cartilage-hairsyndrome; cutis laxa; Turner’s syndrome;Marfan syndrome

TherapyNone; avoidance of surgery, if possible,because of poor-healing wounds

ReferencesGermain DP (2002) Clinical and genetic features

of vascular Ehlers-Danlos syndrome. Annals of Vascular Surgery 16(3):391–397

Elastofibroma

Synonym(s)Elastofibroma dorsi

DefinitionBenign, slow growing, connective tissuetumor, occurring most often in the sub-scapular area in elderly women

PathogenesisPossibly related to trauma by mechanicalfriction of the scapula against the ribs insome cases

Clinical manifestationWell-circumscribed, painless or slightlytender tumor in the subscapular area in eld-erly women

Differential diagnosisLipoma; cyst; leiomyoma; sarcoma; metas-tasis; fibromatosis

TherapySurgical excision in symptomatic patients

ReferencesBieger AK, Varma SK, Timmons MJ (1994)

Elastofibroma dorsi: case report and brief re-view. Annals of Plastic Surgery 32(5):548–549

Elastofibroma dorsi

� Elastofibroma

Elastolysis

� Cutis laxa

Ehlers Danlos syndrome. Marked joint hypermobility of digits


204 Elastolysis cutis laxa

Elastolysis cutis laxa

� Cutis laxa

Elastoma


Elastoma intrapapillare perforans

� Elastosis perforans serpiginosa

Elastoma intrapapillare perforans verruciformis


Elastoma verruciform perforans


Elastosis colloidalis conglomerata


Elastosis intrapapillare


Elastosis perforans


Elastosis perforans serpiginosa

Synonym(s)Elastosis perforans serpiginosum; elastosisintrapapillare; elastoma intrapapillare per-forans; elastoma intrapapillare perforansverruciformis; elastosis perforans; elastomaverruciform perforans; keratosis follicu-laris et parafollicularis serpiginosa;keratosis follicularis serpiginosa; reactiveperforating elastosis

DefinitionSkin condition with abnormal dermal elas-tic tissue fibers and other connective tissueelements expelled via trans-epidermal elim-ination

PathogenesisGranulomatous inflammation displaying anatypical method for removing elastic tissuefrom the area of involvement

Clinical manifestationThree subtypes:Reactive form: associated with other dis-eases such as Down syndrome, Ehlers-Dan-los syndrome, Marfan syndrome, osteogen-esis imperfecta, scleroderma, acrogeria,pseudoxanthoma elasticumDrug-induced form: associated with peni-cillamine useIdiopathic form (most common variety):flesh-colored or pale red, umbilicatedpapules grouped in linear, arciform, circu-lar, or serpiginous patterns; most com-monly occurring over the nape of the neck


Enchondromatosis with multiple cavernous hemangiomas 205

E

Differential diagnosisReactive perforating collagenosis; perforat-ing folliculitis; Kyrle’s disease; folliculitis;prurigo nodularis; granuloma annulare;tinea corporis; lupus erythematosus

TherapyTretinoin; isotretinoin; cryotherapy; elec-trodessication and curettage

ReferencesMehta RK, Burrows NP, Payne CM, Mendelsohn

SS, Pope FM, Rytina E (2001) Elastosis per-forans serpiginosa and associated disorders. Clinical & Experimental Dermatology 26(6):521–524

Elastosis perforans serpiginosum


Elavil

� Amitriptyline

Elephantiasis

DefinitionVisible enlargement of the arms, legs, orgenitals to elephantoid size, usually second-ary to chronic lymphedema

ReferencesMcGuinness CL, Burnand KG (2001) Lymphoede-

ma. Tropical Doctor 31(1):2–7

Elephantiasis nostras verrucosa

DefinitionIn later stages of chronic lymphedema,affected skin becomes indurated and devel-ops verrucous papules and plaques withscale

ReferencesBrantley D, Thompson EC, Brown MF (1995) El-

ephantiasis nostras verrucosa. Journal of the Louisiana State Medical Society 147(7):325–327

Enchondromatosis


Enchondromatosis with multiple cavernous hemangiomas


Elephantiasis nostras verrucosa. Plaque consisting of multiple nodules on the distal lower extremity


206 Endemic pemphigus foliaceus

Endemic pemphigus foliaceus

� Fogo selvagem

Endemic syphilis

� Bejel

Endemic treponematosis

� Pinta

Endep

� Amitriptyline

Endovascular papillary angioendothelioma of childhood

Synonym(s)Dabska tumor; malignant endovascularpapillary angioendothelioma; papillaryintralymphatic angioendothelioma

DefinitionLow-grade angiosarcoma of the skin of chil-dren, with a distinctive histologic architec-ture of anastomosing vascular channelswith intravascular papillary outpouchings

PathogenesisUnclear cell of origin, but tumor markerstudies suggest resemblance to lymphangi-oma

Clinical manifestationSlow-growing, intradermal nodule that isviolaceous, pink, or bluish-black in color

Differential diagnosisReactive angioendotheliomatosis; benignintravascular endothelial hyperplasia; reti-form hemangioendothelioma; glomeruloidhemangioma; infantile hemangioma;Kaposi’s sarcoma; angiolymphoid hyper-plasia

TherapySurgical excision; lymph node dissection ifregional nodes are involved

ReferencesSchwartz RA, Dabski C, Dabska M (2000) The

Dabska tumor: a thirty-year retrospect. Der-matology 201(1):1–5

Enlarged solitary comedone

� Dilated pore

Eosinophilia-myalgia syndrome

Synonym(s)L-tryptophan-induced eosinophilia-myal-gia syndrome; sclerodermoid myalgia;sclerodermoid fasciitis

DefinitionMultisystem disease with prominent eosi-nophilia and generalized myalgia, usuallyassociated with L-tryptophan ingestion

PathogenesisCell-mediated immune response causingwidespread tissue injury; skin and connec-tive tissue fibrosis pervading muscles,nerves, and other organs; L-tryptophaninvolvement in process, but mechanismunclear


Eosinophilic granulomatous vasculitis 207

E

Clinical manifestationAcute episode: shortness of breath, cough,fever; fatigue, arthralgias, paresthesias,severe weakness, muscle cramps, perior-bital and peripheral edema, generalizederythematous eruptionChronic signs and symptoms: generalizedmyalgias, skin tightening; fingers and toesusually spared; Raynaud phenomenon usu-ally absent; scalp alopecia; cutaneoushyperesthesia

Differential diagnosisProgressive systemic sclerosis; toxic oil syn-drome; dermatomyositis; polymyositis;eosinophilic fasciitis; mixed connective dis-ease

TherapyDiscontinuance of all products containingL-tryptophan�; prednisone�

ReferencesBlackburn WD Jr. (1997) Eosinophilia myalgia

syndrome. Seminars in Arthritis & Rheuma-tism 26(6):788–793

Eosinophilic cellulitis

Synonym(s)Wells syndrome; recurrent granulomatousdermatitis with eosinophilia, Wells’ syn-drome, Well’s syndrome

DefinitionCellulitis-like eruption with typical histol-ogy, including flame figures and markeddermal infiltrate of eosinophils

PathogenesisAssociation with insect bites in some cases

Clinical manifestationPruritus and burning sensation, followed bycellulitis-like eruption; large, induratedplaques of edema and erythema, with viola-ceous edges; occasionally also annular

plaques, papules, and urticarial-like wheals;recurrent episodes common

Differential diagnosisCellulitis; erysipelas; urticaria; insect bitereaction; Lyme disease; hypereosinophilicsyndrome; inflammatory metastasis; granu-loma annulare; Churg-Strauss syndrome

TherapyHigh potency topical corticosteroids; pred-nisone

ReferencesWeiss G, Shemer A, Confino Y, Kaplan B, Trau H

(2001) Wells' syndrome: report of a case and re-view of the literature. International Journal of Dermatology 40(2):148–152

Eosinophilic folliculitis

� Eosinophilic pustular folliculitis

Eosinophilic granuloma

� Langerhans cell histiocytosis

Eosinophilic granuloma of soft tissue

� Kimura’s disease

Eosinophilic granulomatous vasculitis



208 Eosinophilic hyperplastic lymphogranuloma

Eosinophilic hyperplastic lymphogranuloma


Eosinophilic lymphofollicular granuloma


Eosinophilic lymphofolliculosis


Eosinophilic lymphoid granuloma


Eosinophilic pustular dermatosis



Synonym(s)Ofuji's disease; Ofuji disease; eosinophilicfolliculitis; HIV-associated eosinophilic fol-

liculitis; HIV-related eosinophilic folliculi-tis, sterile eosinophilic pustulosis;eosinophilic pustular dermatosis; infantile/childhood eosinophilic pustulosis of thescalp

DefinitionRecurrent follicular and non-follicularpapules associated with tissue and periph-eral eosinophilia

PathogenesisUnclear; possibly abnormal immunologicreaction to follicular pathogens

Clinical manifestationFollicular-based erythematous papules andpustules, with or without coalescence intoplaques; face, back, and extensor surfaces ofthe upper extremities most commonlyinvolved in adults; scalp most common sitein children; increased incidence in HIV-infected patients; peripheral eosinophiliaoften present

Differential diagnosisOther forms of folliculitis, including bacte-rial and fungal varieties; pustular psoriasis;acne; rosacea; perioral dermatitis; scabies;candidiasis; folliculitis decalvans; insectbite reaction; Langerhans cell histiocytosis;follicular mucinosis; superficial pemphigus

TherapyDapsone; super potent topical corticoster-oids; prednisone; isotretinoin; itracona-zole; UVB phototherapy; photochemother-apy

ReferencesLazarov A, Wolach B, Cordoba M, Abraham D,

Vardy D (1996) Eosinophilic pustular folliculi-tis (Ofuji disease) in a child. Cutis 58(2):135–138

Ephelides

Synonym(s)Freckles


Epidemic typhus 209

E

DefinitionTan macules which darken after sun expo-sure and fade in the winter months

PathogenesisAutosomal dominant trait; possibly somaticmutations in epidermal melanocytes thatpromote increased melanogenesis

Clinical manifestationMultiple, small, uniformly tan macules onsun-exposed skin; sometimes coalescinginto patches; most common in individualswith fair skin and/or blond or red hair

Differential diagnosisLentigo; seborrheic keratosis; nevus; café aulait spot; tinea versicolor

TherapySun avoidance

ReferencesOrtonne JP (1990) The effects of ultraviolet expo-

sure on skin melanin pigmentation. Journal of International Medical Research 18 Suppl 3:8C–17C

Ephelis ab igne

� Erythema ab igne

Ephidrosis tincta

� Chromhidrosis

Epidemic arthritic erythema

� Rat-bite fever

Epidemic typhus

Synonym(s)Louse-borne typhus; classic typhus

DefinitionAcute, febrile, infectious illness caused byRickettsia prowazekii, characterized byrash, lymphadenopathy, and systemic signsand symptoms

PathogenesisCaused by Rickettsia prowazekii; louseinfected after feeding on rickettsemic per-son with typhus or during a recrudescentcase; bites human to engage in blood mealand causes pruritic reaction on host skin;scratching by host causes crushing of liceand Rickettsia-laden excrement inoculatedinto wound

Clinical manifestationPainless papule at site of chigger bite; sub-sequently undergoes central necrosis withformation of eschar; fever; headache;regional or generalized lymphadenopathy;rigors; myalgias; malaise; CNS symptoms;recrudescent form (Brill-Zinser disease):months to decades after treatment, organ-isms reemerge and cause recurrence oftyphus

Differential diagnosisTularemia; leptospirosis; typhoid fever;other rickettsial infections; viral exanthem;dengue fever; anthrax; ehrlichiosis; infec-tious mononucleosis; Kawasaki disease;malaria; meningococcemia; relapsing fever;toxic shock syndrome; rubella; rubeola

TherapyDoxycycline�; chloramphenicol – 0.5–1 gm IV every 6 hours until 48–72 hoursafter patient becomes afebrile; pediatricdose – 80–100 mg per kg per day IV dividedinto 4 doses until 48–72 hours after patientbecomes afebrile


210 Epidermal cyst

ReferencesBaxter JD (1996) The typhus group. Clinics in

Dermatology 14(3):271–278

Epidermal cyst

� Epidermoid cyst

Epidermal inclusion cyst

� Epidermoid cyst

Epidermal nevus

Synonym(s)Organoid nevus; epithelial nevus

DefinitionCongenital hamartoma of embryonal ecto-dermal origin, classified on the basis of itsmain component, which may be keratinoc-ytic, sebaceous, sweat gland, or follicular

PathogenesisProbable somatic mutation, which mayreflect genetic mosaicism; arises frompluripotential germinative cells of the basal

layer of the embryonic epidermis; possibledermal effect on growth

Clinical manifestationNevus verrucosus (verucous epidermalnevus): usually present at birth or earlychildhood; solitary or multiple, linear or S-shaped, verrucous or velvety plaques, nevercrossing the midline; flexural lesions some-times macerated and foul-smelling; lesionswith sebaceous or apocrine elements mayenlarge at pubertyInflammatory epidermal nevus (ILVN):usually present in the first 5 years of life;pruritic, linear, erythematous, scalyplaques, most commonly on the leg; nevuscomedonicus (comedo nevus): confluentclusters of dilated follicular orifices pluggedwith keratin, giving the appearance ofaggregated open comedones; oftenarranged in a linear, arcuate, or zosteri-form pattern; occasionally paralleling thelines of Voigt or the lines of BlaschkoNevus unius lateris (linear epidermalnevus): solitary linear verrucous plaque,present at birth or in early infancyNevus sebaceous (sebaceous nevus): usu-ally present at birth; well-circumscribed,pink-to-yellow, smooth or velvety plaques,almost always on the head and neck area;enlarges and thickens at puberty; small riskof malignant degeneration to basal cell car-cinomaEpidermal nevus syndrome: one or moreepidermal nevi and involvement of thenervous, ophthalmologic, and/or skeletalsystems; mental retardation, seizures,movement disorders; intracranial and/orintraspinal lipomas

Differential diagnosisProteus syndrome; CHILD syndrome; wart;Darier disease; lichen striatus; incontinen-tia pigmenti; psoriasis; syndrome of Favre-Racouchot; acne vulgaris; mastocytoma;juvenile xanthogranuloma; xanthoma

TherapyNevus verrucosus: surgical excision�;tretinoin; acetretin; inflammatory epider-mal nevus: super potent topical corticoster-

Epidermal nevus. Flesh-colored verrucous nodule on the scalp


Epidermoid cyst 211

E

oids; cryotherapy; surgical excision; nevuscomedonicus: tretinoin; surgical excision;nevus sebaceous: surgical excision�; epi-dermal nevus syndrome: as above for indi-vidual variants

ReferencesLosee JE, Serletti JM, Pennino RP (1999) Epider-

mal nevus syndrome: a review and case report. Annals of Plastic Surgery 43(2):211-214

Epidermodysplasia verruciformis

Synonym(s)None

DefinitionInherited disorder characterized by wide-spread and persistent human papillomavirus (HPV) infection and malignantdegeneration of the virally induced tumors

PathogenesisAutosomal recessive trait; impaired cellularimmunity to specific wart virus subtypes;co-factors: ultraviolet light and X-rays

Clinical manifestationPolymorphic, verrucous or flat-toppedpapules resembling flat warts; macules andreddish-brown plaques with slightly scalysurfaces and irregular borders; localizedmostly on sun-exposed regions, palms,soles, in the axillae, and on external genita-lia; mucous membranes rarely affected;malignant tumors typically appears duringthe fourth and fifth decades of life

Differential diagnosisVerruca plana; squamous cell carcinoma;tinea versicolor; trichoepithelioma; basalcell carcinoma; papular mucinosis; solarelastosis

TherapyCryotherapy; electrodessication and curet-tage; sun avoidance

ReferencesMajewski S, Jablonska S, Orth G (1997) Epidermo-

dysplasia verruciformis. Immunological and nonimmunological surveillance mechanisms: role in tumor progression. Clinics in Dermatol-ogy 15(3):321–334

Epidermoid carcinoma

� Squamous cell carcinoma

Epidermoid cyst

Synonym(s)Epidermal cyst; epidermal inclusion cyst;wen; atheroma; steatoma; sebaceous cyst

DefinitionCyst with a stratified squamous lining,which produces keratin

PathogenesisDerived from follicular infundibulum; oftenoccurring at site of previous trauma(inflammatory acne, etc.)

Clinical manifestationWhite or pale yellow, deep dermal or sub-cutaneous, medium-firm papule or nodule,often with a central pore; cheesy, foul-smelling material sometimes exuded withlateral pressure

Differential diagnosisLipoma; trichilemmoma; steatocystomamultiplex; granuloma annulare; sarcoido-sis; lymphocytic infiltrates; insect bite reac-tion; acquired perforating disease; metasta-sis


212 Epidermolysis bullosa

TherapySimple excision by sharp dissection�; ellip-tical excision; marsupialization of largelesions; inflamed lesion: incision and drain-age of purulent material; triamcinolone (3–5 mg per ml) injected intralesionally



Epidermolysis bullosa

Synonym(s)None

DefinitionGroup of inherited disorders characterizedby blister formation in response to mechan-ical trauma

PathogenesisEpidermolysis bullosa simplex: associatedwith mutations of the genes coding forkeratins 5 and 14; level of skin separation atthe mid basal cell associated with variableintermediate filament clumpingJunctional epidermolysis bullosa: muta-tions in genes coding for laminin 5 subu-

nits (α3 chain, laminin β3 chain, laminin γ2chain), collagen XVII (BP180), α6 integrin,and β4 integrinDystrophic epidermolysis bullosa: muta-tions of the gene coding for type VII colla-gen (COL7A1); anchoring fibrils affected;degree of involvement ranging from subtlechanges to complete absence

Clinical manifestationEpidermolysis bullosa simplex:• Weber-Cockayne variant: most commonform; blisters usually precipitated by trau-matic event; most frequently occurring onthe palms and soles, often with hyperhidro-sis• Severe variant: generalized onset of blis-ters occurring at or shortly after birth;hands, feet, and extremities most commonsites of involvement• Koebner variant: sometimes has palmo-plantar hyperkeratosis and erosions• Dowling-Meara variant: involves oral mu–cosa with grouped herpetiform blisters.Junctional epidermolysis bullosa:• Letalis (Herlitz) variant: generalized blis-tering at birth; orificial erosions around themouth, eyes, and nares; often accompaniedby significant hypertrophic granulation tis-sue; involvement of the corneal, conjuncti-val, tracheobronchial, oral, pharyngeal,esophageal, rectal, and genitourinarymucosal surfaces; internal complications:hoarse cry, cough, and other respiratorydifficulties; poor prognosis• Nonlethal junctional variant (mitis form):usually survives infancy; generalized blis-tering; improves with age; scalp, nail, andtooth abnormalities; periorificial erosionsand hypertrophic granulation tissue;mucous membranes erosions, resulting instrictures.Dystrophic epidermolysis bullosa:• Dominantly inherited variant; onset ofdisease usually at birth or during infancy;generalized blistering is common presenta-tion; evolution to localized blistering withage

Epidermolysis bullosa. Bullae, erosions, and scarring of the hands


Epidermolysis bullosa acquisita 213

E

• Cockayne-Touraine variant: acral distri-bution and minimal oral or tooth involve-ment• Pasini variant: more extensive blistering,scarlike papules on the trunk (albopapu-loid lesions); involvement of the oralmucosa and teeth; dystrophic or absentnails common• Mitis variant: involves acral areas andnails with little mucosal involvement; clini-cal manifestations similar to the domi-nantly inherited forms• Severe recessive variant (Hallopeau-Sie-mens): generalized blistering at birth; sub-sequent extensive dystrophic scarring, mostprominent on the acral surfaces, some-times resulting in pseudosyndactyly (mit-ten-hand deformity) of the hands and feet;flexion contractures of the extremitiesincreasingly common with age; dystrophyof nails and teeth; involvement of internalmucosa sometimes resulting in esophagealstrictures and webs, urethral and anal sten-osis, phimosis, and corneal scarring; intes-tinal malabsorption leading to a mixed ane-mia resulting from a lack of iron absorp-tion and failure to thrive; significant risk ofdeveloping aggressive squamous cell carci-nomas in areas of chronic erosions

Differential diagnosisLinear IgA bullous disease; bullous pemphi-goid; epidermolysis bullosa acquisita; fric-tion blisters; pemphigus vulgaris; burn

TherapyAvoidance of frictional trauma�; carefulattention to skin and dental hygiene�;severe disease: soft diet to prevent esopha-geal trauma and blistering; skin equivalentdressings to promote epithelialization

ReferencesFine JD, Eady RA, Bauer EA, Briggaman RA,

Bruckner-Tuderman L, et al. (2000) Revised classification system for inherited epidermoly-sis bullosa: report of the Second International Consensus Meeting on diagnosis and classifi-cation of epidermolysis bullosa. Journal of the American Academy of Dermatology 42(6):1051–1066


Synonym(s)Acquired epidermolysis bullosa; dermolyticpemphigoid

DefinitionChronic autoimmune blistering disease,with lesions often occurring at sites oftrauma

PathogenesisIgG autoantibodies specific for anchoringfibrils (type VII collagen) of the skin base-ment membrane causes an inflammatoryprocess which is a contributing factor toblister formation; skin trauma a contribut-ing factor; genetic factors possibly impor-tant, since HLA-DR2 is overrepresented inthose with this condition

Clinical manifestationNon-inflammatory bullae at sites of minorskin trauma, which heal with scars and/ormilia; widespread inflammatory bullae notrelated to trauma; mucous membrane blis-ters and erosions, leading to scarring

Differential diagnosisEpidermolysis bullosa; bullous pemphig-oid; cicatricial pemphigoid; linear IgA bul-lous dermatosis; bullous lupus erythemato-sus; porphyria cutanea tarda; bullous dis-ease of diabetes mellitus; erythemamultiforme

TherapyPrednisone 1 mg per kg PO daily�; corti-costeroid sparing agents – azathioprine;methotrexate; mycophenolate mofteil;cyclophosphamide; dapsone

ReferencesKirtschig G, Murrell D, Wojnarowska F, et al.

(2002) Interventions for mucous membrane pemphigoid/cicatricial pemphigoid and epi-dermolysis bullosa acquisita: A systematic lit-


214 Epidermolysis bullosa dystrophica

erature review. Archives of Dermatology 138:380–384

Epidermolysis bullosa dystrophica


Epidermolysis bullosa, Herlitz variant


Epidermolysis bullosa herpetiformis


Epidermolysis bullosa letalis


Epidermolysis bullosa simplex



Synonym(s)Bullous congenital ichthyosiform erythro-derma; bullous ichthyotic erythroderma;

ichthyosis bullosa of Siemens; ichthyosishystrix of Curth-Macklin

DefinitionCongenital ichthyosis with characteristichistologic finding of epidermolytic hyperk-eratosis

PathogenesisAutosomal dominant trait; defect in thegenes for keratin 1 and keratin 10

Clinical manifestationPresents at birth or shortly thereafter aserythema, blistering, and/or scaling;marked hyperkeratosis shortly after birth;scales are small, dark, with corrugatedappearance; scales sometimes shedand,reaccumulate; keratotic skin in intertrigi-nous areas which may become maceratedand foul smelling; blisters occur in crops,rupturing, and leaving red, painful,denuded base; bullae tend to disappearbefore age 20; NPS subtype – lacks severepalmoplantar involvement; PS subtype –severe palmoplantar involvement; no ectro-pion

Differential diagnosisNon-bullous ichthyosiform erythroderma;lamellar ichthyosis; X-linked ichthyosis;epidermolysis bullosa; incontinentia pig-menti; bullous impetigo; staphylococcalscalded skin syndrome

TherapyPrednisone; beta carotene; acetretin; tretin-oin; alpha-hydroxy acid

ReferencesBale SJ, Compton JG, DiGiovanna JJ (1993) Epi-

dermolytic hyperkeratosis. Seminars in Der-matology 12(3):202–209

Epiloia



Erysipelas 215

E

Epithelial nevus

� Epidermal nevus

Epithelioid angiomatosis


Epithelioid hemangioma

� Angiolymphoid hyperplasia with eosinophilia

Epithelioma adenoides cysticum


Epithelioma contagiosum

� Molluscum contagiosum

Epithelioma cuniculatum

� Verrucous carcinoma

Erosio interdigitalis blastomycetica

� Candidiasis

Erosive adenomatosis of the nipple

Synonym(s)Benign papillomatosis of the nipple; floridpapillomatosis of the nipple; papillary ade-noma of the nipple; subareolar adenomato-sis; papillomatosis of the subareolar ducts

DefinitionBenign tumor of the nipple, with apocrinedifferentiation

PathogenesisHamartomatous proliferation of the lactif-erous ducts

Clinical manifestationAsymptomatic-to-slightly-pruritic, unilat-eral, eroded, crusted plaque on the nipple;nipple discharge sometimes occurs pre-menstrually

Differential diagnosisPaget’s disease of the breast; contact derma-titis; basal cell carcinoma; apocrine glandtumors; hidradenitis suppurativa

TherapyExcision of the nipple and subareolar tis-sue�

ReferencesMontemarano AD, Sau P, James WD (1995) Super-

ficial papillary adenomatosis of the nipple: a case report and review of the literature. Journal of the American Academy of Dermatology 33(5 Pt 2):871–875

Erysipelas

Synonym(s)None


216 Erysipeloid

DefinitionSkin infection involving the dermis andlocal lymphatics, usually caused by group Abeta-hemolytic streptococci

PathogenesisBacterial infection, typically caused bygroup A Streptococcus

Clinical manifestationAbrupt onset of illness with fever and chills,muscle and joint pain, nausea, headache;skin change begins as small erythematouspatch and progresses to red, indurated,shiny plaque; raised, sharply demarcated,advancing margins, with skin warmth,edema, and tenderness; lymphatic involve-ment with overlying skin streaking andregional lymphadenopathy

Differential diagnosisContact dermatitis; seborrheic dermatitis;lupus erythematosus; angioedema; herpeszoster; erysipeloid; necrotizing fasciitis

TherapyPenicillin G procaine; Penicillin VK; dicola-cillin if staphyloccocal infection present;cephalexin if patient is allergic to penicillin

ReferencesChartier C, Grosshans E (1996) Erysipelas: an up-

date. International Journal of Dermatology 35(11):779–781

Erysipeloid

Synonym(s)Erysipeloid of Rosenbach

DefinitionAcute bacterial infection of traumatizedskin caused by the microorganism Erysip-elothrix rhusiopathiae (insidiosa)

PathogenesisCausative organism, E. rhusiopathiae,enters the skin through scratches or pricks;organism produces enzymes that help itdissect through the tissues; inflammationproduced when immune system activatedagainst foreign antigen

Clinical manifestationFood handlers (home makers, farmers, fish-ermen, and butchers) at increased risk ofacquiring the infectionLocalized form: well demarcated, bright-red-to-purple, warm, tender plaques with asmooth, shiny surface, most commonly onthe handsDiffuse cutaneous form: multiple, welldemarcated, violaceous plaques with anadvancing border and central clearingSystemic form: localized areas of swellingsurrounding a necrotic center; sometimespresenting as follicular, erythematouspapules; endocarditis as complication ofsepticemia

Differential diagnosisCellulitis; erysipelas; fixed medication reac-tion; erythema nodosum; leishmaniasis

TherapyPenicillin�

ReferencesReboli AC, Farrar WE (1989) Erysipelothrix rhusi-

opathiae: an occupational pathogen. Clinical Microbiology Reviews 2(4):354–359

Erysipelas. Erythematous, edematous plaque on the central face


Erythema annulare centrifugum 217

E

Erysipeloid of Rosenbach

� Erysipeloid

Erythema à calore


Erythema ab igne

Synonym(s)Erythema ab igne elastosis; ephelis ab igne;erythema à calore; toasted skin syndrome

DefinitionChanges in the skin caused by chronic andrepeated exposure to infrared radiation

PathogenesisUnclear mechanism; repeated external heatexposure in the range of 43–47°C resultingin histopathologic changes similar to thoseseen in solar-damaged skin

Clinical manifestationReticulated violaceous and hyperpig-mented plaques, most common on the legsof women; poikiloderma occurs with severelong-standing disease

Differential diagnosisLivedo reticularis; poikiloderma of Civatte;poikiloderma atrophicans vasculare; mor-phea; livedo vasculitis

TherapyNd:YAG, ruby, or alexandrite laser

ReferencesPage EH, Shear NH (1988) Temperature-depend-

ent skin disorders. Journal of the American Academy of Dermatology 18(5 Pt 1):1003–1019

Erythema ab igne elastosis



Synonym(s)Erythema gyratum perstans; erythema exu-dativum perstans; erythema marginatumperstans; erythema perstans; erythema fig-uratum perstans; erythema microgyratumperstans; erythema simplex gyratum;erythema perstans

DefinitionFigurate erythema with a characteristicadvancing, scaly margin and central clear-ing

PathogenesisProbably represents hypersensitivity reac-tion to a variety of agents, including drugs,arthropod bites, infections (bacterial,mycobacterial, viral, fungal, filarial), ingest-ants (blue cheese penicillium), and malig-nancy

Clinical manifestationBegins as asymptomatic, erythematouspapules which spread peripherally whileclearing centrally; often a trailing scale onthe inner aspect of the advancing edge;appears on any skin surface other than thepalms and soles; may be associated with anunderlying disease (e.g., infection, malig-nancy, or other systemic illness)

Differential diagnosisErythema marginatum rheumaticum; ery-thema migrans; erythema gyratum repens;urticaria; granuloma annulare; sarcoidosis;tinea corporis; seborrheic dermatitis; lupuserythematosus; benign lymphocytic infil-trate; rheumatoid arthritis; psoriatic arthri-


218 Erythema areata migrans

tis; lupus erythematosus; Reiter syndrome;gonococcal arthritis

TherapyPrednisone; high potency topical corticos-teroids

ReferencesTyring SK (1993) Reactive erythemas: erythema

annulare centrifugum and erythema gyratum repens. Clinics in Dermatology 11(1):135–139

Erythema areata migrans

� Benign migratory glossitis

Erythema chronicum figuratum melanodermicum

� Ashy dermatosis

Erythema circinata


Erythema contusiformis


Erythema craquelé

DefinitionManifestation of dry skin with large dryscales and fine fissures giving a cracked-potappearance



Erythema dyschromicum perstans

� Ashy dermatosis

Erythema dyschronicum perstans

� Ashy dermatosis

Erythema dyspepsicum

� Erythema toxicum

Erythema elevatum diutinum

Synonym(s)Extracellular cholesterosis

DefinitionLeukocytoclastic vasculitis characterized byred, purple, brown, or yellow papules,plaques, or nodules

PathogenesisPossibly involves immune complex deposi-tion with subsequent inflammatory cas-cade; associated with IgA monoclonal gam-mopathy, recurrent bacterial infections,(especially streptococcal), viral infections(including Hepatitis B or HIV), and rheu-matologic disease


Erythema induratum 219

E

Clinical manifestationRed, violaceous, smooth, brown or yellowpapules, plaques, or nodules over extensorsurfaces, especially over the joints; occa-sional crusting or bleeding

Differential diagnosisAcute bebrile neutrophilic dermatosis;granuloma annulare; insect bite reaction;sarcoidosis; rheumatoid nodules; goutytophi; multicentric reticulohistiocytosis;xanthomas; erythema multiforme

TherapyDapsone�

ReferencesGibson LE, el-Azhary RA (2000) Erythema eleva-

tum diutinum. Clinics in Dermatology 18(3):295–299

Erythema exudativum

� Erythema multiforme

Erythema exudativum perstans

� Erythema annulare centrifugum

Erythema figuratum perstans


Erythema gyratum perstans


Erythema gyratum repens

Synonym(s)None

DefinitionFigurate erythema with a distinctive clini-cal appearance, which serves as a marker ofinternal malignancy

PathogenesisPossibly involves a cutaneous response totumor antigens

Clinical manifestationEruption often precedes diagnosis ofunderlying malignancy; wood-grainappearance created by concentric, pruritic,mildly scaling bands of flat-to-slightly-pal-pable erythema, with rapid migration of thebands; course of eruption mirrors course ofthe underlying illness, with clearance ofrash and relief of pruritus within 6 weeks ofunderlying illness resolution; sites of predi-lection trunk and extremities

Differential diagnosisErythema annulare centrifugum; granu-loma annulare; tinea corporis; sarcoidosis;lupus erythematosus; glucagonoma syn-drome; urticaria

TherapyTreatment of underlying malignancy; pred-nisone; high potency topical corticosteroids

ReferencesEubanks LE, McBurney E, Reed R (2001) Ery-

thema gyratum repens. American Journal of the Medical Sciences 321(5):302–305

Erythema induratum

� Nodular vasculitis


220 Erythema infectiosum

Erythema infectiosum

Synonym(s)Fifth disease, slapped-cheek disease,academy rash, Sticker's disease, Sticker dis-ease

DefinitionChildhood exanthem caused by human Par-vovirus B19, in which a 3-phased cutaneouseruption follows a mild prodrome

PathogenesisParvovirus B19 viremia; production of spe-cific immunoglobulin M (IgM) antibodiesand subsequent formation of immune com-plexes; clinical findings probably resultfrom the deposition of the immune com-plexes in the skin and joints

Clinical manifestation4–14 day incubation period; virus spreadsprimarily via aerosolized respiratory drop-letsMild prodromal phase, including head-ache, coryza, low-grade fever, pharyngitis,and malaiseFirst stage: erythema of the cheeks, withnasal, perioral, and periorbital sparing(slapped-cheek appearance) and fades over2–4 daysSecond stage: within 1–4 days of the facialrash, erythematous macular-to-morbilli-form eruption occurs primarily on theextremitiesThird stage: after several days, most of thesecond stage eruption fades into a lacy pat-tern, particularly on the proximal extremi-ties; lasts from 3 days to 3 weeks; after start-ing to fade, exanthem sometimes recursover several weeks following exercise, sunexposure, friction, bathing in hot water, orstress; adults sometimes develop polyar-thropathy

Differential diagnosisOther viral exanthems; medication reac-tion; Lyme disease; lupus erythematosus;

acute rheumatic fever; allergic hypersensi-tivity reaction

TherapyAntihistamines, first generation, as seda-tive and mild anti-pruritic agent

ReferencesKoch WC (2001) Fifth (human parvovirus) and

sixth (herpesvirus 6) diseases. Current Opin-ion in Infectious Diseases 14(3):343–356

Erythema marginatum

DefinitionSuperficial, often asymptomatic, form ofgyrate erythema, characterized by a tran-sient eruption of macular to slightly palpa-ble, non-scaling plaques on the trunk andextensor surfaces of the extremities; associ-ated with rheumatic fever

ReferencesRullan E, Sigal LH (2001) Rheumatic fever. Cur-

rent Rheumatology Reports 3(5):445–452

Erythema marginatum perstans


Erythema microgyratum perstans


Erythema migrans

� Lyme disease


Erythema neonatorum 221

E

Erythema multiforme

Synonym(s)Erythema exudativum; Hebra’s disease;erythema polymorphe

DefinitionAcute inflammatory disorder related tonumerous factors, characterized by distinc-tive clinical eruption, with hallmark of irisor target lesion

PathogenesisUnclear; herpes-associated disease appearsto represent the result of a cell-mediatedimmune reaction associated with herpessimplex virus (HSV) antigen

Clinical manifestationMost commonly associated with herpessimplex virus infection; also associatedwith other infections, drug ingestion, rheu-matic diseases, vasculitides, non-Hodgkin’slymphoma, leukemia, multiple myeloma,myeloid metaplasia, polycythemiaErythema multiforme minor variant: occa-sional mild flu-like prodrome; initial lesiondull red macule or urticarial plaque in thecenter, with small papule, vesicle, or bullasometimes developing; raised, pale ringwith edematous; periphery gradually

becoming violaceous and forming concen-tric target lesion; lesions appear predomi-nantly on the extensor surfaces of acralextremities and spread centripetally; milderosions of one mucosal surface; palms,neck, and face frequently involvedErythema multiforme major variant: pro-drome of moderate fever, general discom-fort, cough, sore throat, vomiting, chestpain, and diarrhea, usually for 1–14 dayspreceding the eruption; skin lesions sameas with erythema multiforme minor; severeerosions of at least 2 mucosal surfaces; gen-eralized lymphadenopathy

Differential diagnosisStevens-Johnson syndrome; toxic epider-mal necrolysis; Henoch-Schönlein pur-pura; urticaria; viral exanthem; Kawasakidisease; figurate erythema; fixed drug erup-tion; lupus erythematosus; primary her-petic gingivostomatitis; Behçet’s disease;aphthous stomatitis

TherapyAntihistamines, first generation; pred-nisone; herpes simplex virus prophylaxiswith valacyclovir, if more than 4–5 episodesper year

ReferencesSalman SM, Kibbi AG (2002) Vascular reactions in

children. Clinics in Dermatology 20(1):11–15

Erythema multiforme major

� Stevens-Johnson syndrome

Erythema neonatorum


Erythema multiforme. Target-like papules on the palm


222 Erythema neonatorum allergicum

Erythema neonatorum allergicum


Erythema nodosum

Synonym(s)Dermatitis contusiformis; erythema con-tusiformis; focal septal panniculitis; nodosefever

DefinitionInflammatory vascular reaction pattern tomultiple causes; characterized by tendersubcutaneous nodules, usually on the ante-rior legs

PathogenesisProbably is delayed hypersensitivity reac-tion to a variety of antigens; most commonassociations with streptococcal infectionsin children and sarcoidosis in adults; otherassociations include tuberculosis, myco-plasma pneumonia, leprosy, coccidioid-omycosis, North American blastomycosis,histoplasmosis, inflammatory bowel dis-ease, pregnancy, and Behçet’s disease; asso-ciated medications include oral contracep-tives and sulfonamides

Clinical manifestationProdrome of flulike symptoms of fever andgeneralized aching; lesions begin as poorly-defined, red, tender nodules; become firmand painful during the second week; some-times becoming fluctuant; not suppuratingor ulcerating; individual lesions lastapproximately 2 weeks; associated legedema and pain

Differential diagnosisNodular vasculitis; insect bite reaction; ery-sipelas; cellulitis; superficial thrombophle-

bitis; Weber-Christian disease; pancreaticpanniculitis; lupus profundus; traumaticpanniculitis; polyarteritis nodosa; rheuma-toid nodules

TherapyNon-steroidal anti-inflammatory agents;bed rest; leg elevation; prednisone

ReferencesRequena L, Requena C (2002) Erythema nodo-

sum. Dermatology Online Journal 8(1):4

Erythema nodosum migrans


Erythema nuchae

� Salmon patch

Erythema papulosum


Erythema perstans

� Ashy dermatosis� Erythema annulare centrifugum

Erythema polymorphe

� Erythema multiforme


Erythrasma 223

E

Erythema simplex gyratum


Erythema solare

� Sunburn

Erythema toxicum

Synonym(s)Erythema toxicum neonatorum; erythemaneonatorum; toxic erythema; erythemaneonatorum allergicum; erythema papulo-sum; urticaria neonatorum; erythema dys-pepsicum

DefinitionBenign, self-limited eruption occurring pri-marily in healthy newborns in the earlyneonatal period

PathogenesisUnknown

Clinical manifestationUsual onset within the first 4 days of life infull-term infants, with peak onset occur-ring within the first 48 hours followingbirth; presents with a blotchy, evanescent,macular erythema, often on the face ortrunk; sites of predilection include the fore-head, face, trunk, and proximal extremi-ties; mucous membranes usually spared

Differential diagnosisCandidiasis; miliaria; pyoderma; insect bitereaction; varicella; herpes simplex virusinfection; urticaria; folliculitis; transientneonatal pustular melanosis

TherapyNone

ReferencesWagner A (1997) Distinguishing vesicular and

pustular disorders in the neonate. Current Opinion in Pediatrics 9(4):396–405

Erythema toxicum neonatorum


Erythemato-papulous acrodermatitis


Erythemato-vesiculo-papulous eruptive syndrome


Erythermalgia

� Erythromelalgia

Erythrasma

Synonym(s)None

DefinitionChronic superficial infection of the inter-triginous areas caused by Corynebacte-rium minutissimum


224 Erythroderma

PathogenesisUnder favorable conditions, such as heatand humidity, Corynebacteria organismsproliferate and cause clinical signs

Clinical manifestationWell demarcated, brown-red, minimallyscaly plaques, commonly occurring overinner thighs, crural region, scrotum, andtoe webs; other intertriginous sites such asaxillae, submammary area, periumbilicalregion, and intergluteal fold less commonlyinvolved; toe web lesions appear macer-ated; predisposing factors: excessive sweat-ing and hyperhidrosis, disrupted cutane-ous barrier, obesity, diabetes mellitus, andimmunocompromised state

Differential diagnosisTinea pedis; tinea corporis; tinea cruris;contact dermatitis; dyshidrotic eczema;intertrigo; contact dermatitis

TherapyErythromycin base; clarithromycin; dryingpowder applied twice daily

ReferencesHoldiness MR (2002) Management of cutaneous

erythrasma. Drugs 62(8):1131–41

Erythroderma

� Exfoliative dermatitis

Erythroderma exfoliativa recidivans faciei

� Riehl’s melanosis

Erythrohepatic protoporphyria


Erythrokeratoderma

� Erythrokeratodermia variabilis

Erythrokeratodermia figurata variabilis


Erythrokeratodermia papillaris et reticularis


Erythrokeratodermia progressiva symmetrica

� Progressive symmetric keratoderma

Erythrokeratodermia variabilis

Synonym(s)Erythrokeratoderma; keratosis rubra figu-rata; erythrokeratodermia figurata variabi-lis


Erythromelalgia 225

E

DefinitionDisorder of cornification associated withtransient noninflammatory erythema andpersistent, but changing, scaliness

PathogenesisMutations identified in the connexin geneGJB3; possibly caused by impaired gapjunctional intercellular communication dueto a defect in gap junctions

Clinical manifestationTransient, circumscribed, highly variable,figurate erythematous patches, sometimessurrounded by a hypomelanotic halo,involving any part of the skin; lesions mostprevalent during childhood and sometimesbecoming less frequent as the patient ages;burning sensation sometimes preceding oraccompanying erythema; variably chang-ing, brownish, hyperkeratotic plaques withgeographic borders, symmetrically distrib-uted over the limbs, buttocks, and trunk;flexures, face, and scalp usually spared

Differential diagnosisProgressive symmetric erythrokeratoder-mia; Giroux-Barbeau erythrokeratodermiawith ataxia; Greither disease; erythrokera-tolysis hiemalis; ichthyosis linearis circum-flexa; psoriasis; mycosis fungoides; lupuserythematosus; lamellar ichthyosis; gyrateerythema; atopic dermatitis

TherapyAcitretin�; emollients and/or keratolytics,such as alpha hydroxy acids

ReferencesHendrix JD Jr, Greer KE (1995) Erythrokeratoder-

mia variabilis present at birth: case report and review of the literature. Pediatric Dermatology 12(4):351–354

Erythrokeratolysis hiemalis

� Keratolytic winter erythema

Erythromelalgia

Synonym(s)Erythermalgia

DefinitionDisorder characterized by paroxysmalburning pain, warmth, and redness of theextremities

PathogenesisUnclear; arteriolar fibrosis and occlusionwith platelet thrombi often present; pros-taglandins and cyclooxygenase involved

Clinical manifestationMost cases primary (idiopathic); secondaryform sometimes precede myeloprolifera-tive disorder with thrombocytosis; dra-matic relief with aspirin typical of this typeand useful in diagnosis; painful, warmextremities brought on by warming ordependency, lasting minutes to days, andrelieved by cooling; lower extremitiesaffected more often than upper extremities;symptoms worsening with warming ofextremity or placing of extremity in adependent position; symptoms sometimesdecrease with cooling and elevation ofextremity; no symptoms or signs betweenattacks

Differential diagnosisRaynaud phenomenon; reflex sympatheticdystrophy; cellulitis; vasculitis; frostbite

TherapyCooling or elevating extremity to relievesymptoms of an attack�; aspirin 500 mg POas needed; chemotherapy for myeloprolifer-ative disorder

ReferencesCohen JS (2000) Erythromelalgia: new theories

and new therapies. Journal of the American Academy of Dermatology 43(5 Pt 1):841–847


226 Erythromycin, systemic

Erythromycin, systemic

Trade name(s)Eryc; E-mycin; PCE; EES; Ilosone



Mechanism of actionInhibition of RNA-dependent protein syn-thesis by binding to the 50S subunit of theribosome

Dosage form250 mg, 333 mg, 400 mg, 500 mg tablet


Common side effectsCutaneous: urticaria or other vascular reac-tion, stomatitisGastrointestinal: nausea and vomiting,diarrhea, abdominal cramps, jaundiceLaboratory: elevated liver enzymes; eosi-nophilia

Serious side effectsBone marrow: suppressionCardiovascular: arrhythmias, hypotensionCutaneous: anaphylaxis, Stevens-Johnsonsyndrome

Drug interactionsAmiodarone; amitriptyline; budesonide;buspirone; carbamazepine; clozapine; oralcontraceptives; cyclosporine; digoxin; ergotalkaloids; methadone; phenytoin; pimoz-ide; protease inhibitors; quinidine; statins;tacrolimus; theophylline; valproic acid;vinca alkaloids; warfarin

Contraindications/precautionsHypersensitivity to drug class or compo-nent; caution in patients with myastheniagravis or impaired liver function



Erythromycin, topical

Trade name(s)Emgel; Erycette; EryDerm; Erymax;Erythra-Derm; T-Stat; Theramycin; Staticin


Drug classTopical macrolide antibiotic; anti-inflam-matory

Mechanism of actionInhibition of RNA-dependent protein syn-thesis by binding to the 50S subunit of theribosome

Dosage form2% gel; 1.5, 2% solution


Common side effectsCutaneous: burning sensation, dryness,peeling, pruritus, erythema


Drug interactionsTopical clindamycin


Erythromycin, topical 227

E

Erythromycin, systemic. Dermatologic indications and dosage


Acne vulgaris 250–1000 mg PO daily 250–500 mg PO daily


500 mg PO 4 times daily for 10 days 30–50 mg per kg daily divided into 4 doses for 10 days

Bacillary angiomatosis 500 mg PO 4 times daily for 3 weeks 30–50 mg per kg daily divided into 4 doses for 4 weeks

Bartonellosis 500 mg PO 4 times daily for 3 weeks 30–50 mg per kg daily divided into 4 doses for 10 days

Bejel 500 mg PO 4 times daily for 15 days 8 mg per kg daily divided into 4 doses for 15 days

Ecthyma 250–500 mg PO 4 times daily for 10 days

30–50 mg per kg daily divided into 4 doses for 10 days

Erythrasma 500 mg PO 4 times daily for 7–10 days

30–50 mg per kg daily divided into 4 doses for 7–10 days


500 mg PO twice daily 30–50 mg per kg daily divided into 2 doses



Leptospirosis 250–500 mg PO 4 times daily for 3 weeks

30–50 mg per kg daily divided into 4 doses for 3 weeks

Lyme disease 500 mg PO 4 times daily for 3 weeks 30–50 mg per kg daily divided into 4 doses for 10 days


500 mg PO 4 times daily for 3 weeks 30–50 mg per kg daily divided into 4 doses for 3 weeks

Perioral dermatitis 250–500 mg PO twice daily for at least 30 days

125–250 mg PO 4 times daily for at least 30 days

Pinta 500 mg PO 4 times daily for 15 days 8 mg per kg daily divided into 4 doses for 15 days

Pitted keratolysis 250–500 mg PO 4 times daily for 10 days


Pityriasis lichenoides 500 mg PO twice daily 30–50 mg per kg daily divided into 2 doses

Pityriasis rosea 500 mg PO 4 times daily for 2 weeks 30–50 mg per kg daily divided into 4 doses for 2 weeks

Relapsing fever (louse-borne)

500 mg PO for 1 dose 250 mg PO for 1 dose

Relapsing fever (tick-borne)

500 mg PO 4 times daily for 7 days 30–50 mg per kg daily divided into 4 doses for 7days

Rosacea 250–500 mg PO twice daily for at least 30 days

125–250 mg PO 4 times daily for at least 30 days

Scarlet fever 500 mg PO 4 times daily for 7–10 days


Syphilis 500 mg PO 4 times daily for 2–4 weeks

30–50 mg per kg daily divided into 4 doses for 2–4 weeks


228 Erythroplasia of Queyrat

Contraindications/precautionsHypersensitivity to drug class or compo-nent; caution about resistant organismswhen used without benzoyl peroxide

ReferencesGreenwood R, Burke B, Cunliffe WJ (1986) Evalu-

ation of a therapeutic strategy for the treatment of acne vulgaris with conventional therapy. British Journal of Dermatology 114(3):353–358

Erythroplasia of Queyrat

Synonym(s)Carcinoma in situ of the penis

DefinitionPrecancerous epithelial proliferation of thepenis, almost always occuring in uncircum-cised men

PathogenesisArises from squamous epithelial cells of theglans penis or inner lining of prepuce; mul-tiple contributing factors including chronicirritation (urine, smegma), inflammation(heat, friction, maceration) and infection(herpes simplex virus infection, humanpapillomavirus infection)

Clinical manifestationMinimally raised, erythematous plaques,which may be smooth, velvety, scaly,crusted, or verrucous; ulceration or dis-tinct papillomatous papules suggest pro-gression to invasive squamous cell carci-noma

Differential diagnosisBalanitis of Zoon; balanitis xerotica obliter-ans; candidiasis; contact dermatitis; squa-mous cell carcinoma; fixed drug reaction;psoriasis; lichen planus

TherapyFluorouracil, topical; destruction by liquidnitrogen cryotherapy or electrodesiccationand curettage

ReferencesFitzgerald DA (1998) Cancer precursors. Semi-

nars in Cutaneous Medicine & Surgery 17(2):108–113

Erythroplasia of Zoon

� Zoon balanitis

Trench fever 500 mg PO 4 times daily for 4 weeks 30–50 mg per kg daily divided into 3 doses for 15 days

Yaws 500 mg PO 4 times daily for 15 days 30–50 mg per kg daily divided into 4 doses for 15 days

Erythromycin, systemic. Dermatologic indications and dosage (Continued)


Erythroplasia of Queyrat. Ill-defined, scaly, eroded plaque of the glans penis


Essential lipoid histiocytosis 229

E

Erythropoietic porphyria


Erythropoietic protoporphyria

Synonym(s)Erythrohepatic protoporphyria; congenitalerythropoietic protoporphyria; protopor-phyria

DefinitionInherited disorder of porphyrin-hememetabolism caused by mutations in thegene encoding ferrochelatase, resulting inaccumulation of excess protoporphyrin thatmediates a distinctive form of cutaneousphotosensitivity

PathogenesisMutations of the ferrochelatase gene, lead-ing to excess protoporphyrin, a moleculecapable of transformation to excited statesby absorption of light energy; photoxida-tive damage to biomolecular targets in theskin, resulting in immediate phototoxicsymptoms

Clinical manifestationImmediate edema, erythema, and petechiaeafter sun exposure; occasional vesicles;chronic skin changes, including facial scars,

perioral furrowing, and aged-appearing,thickened, or hyperkeratotic skin of thedorsal hands; with sustained, more intense,or frequent exposures, waxy scleroderma-like induration and/or weather-beaten orcobblestone textures of the face and dorsalaspects of hands; progressive liver failure inrare instances, with hepatosplenomegalyand jaundice

Differential diagnosisSolar urticaria; acute tar photosensitivity;hereditary coproporphyria; porphyria cuta-nea tarda; pseudoporphyria; polymor-phous light eruption; light-sensitive atopicdermatitis

TherapyBeta-carotene 120–300 mg PO per day; sunavoidance�

ReferencesMurphy GM (1999) The cutaneous porphyrias: a

review. The British Photodermatology Group. British Journal of Dermatology 140(4):573–581

Erythropoietic uroporphyria


Essential lipoid histiocytosis


Erythromycin, topical. Dermatologic indications and dosage



Familial benign chronic pemphigus


Perioral dermatitis Apply twice daily Apply twice daily

Pitted keratolysis Apply twice daily for 2–4 weeks Apply twice daily for 2–4 weeks



230 Essential melanotic pigmentation

Essential melanotic pigmentation

� Laugier-Hunziger syndrome

Essential mixed cryoglobulinemia


Etat craquelé

� Asteatotic eczema

Eumycetoma

Synonym(s)Madura foot; maduromycosis; fungal myce-toma; eumycotic mycetoma; melanoid myc-etoma; ochroid mycetoma

DefinitionChronic cutaneous and subcutaneous infec-tion caused by various genera of fungi andcharacterized by progressive destruction ofsoft tissue

Pathogen,esisInfectious agents primarily saprophyticmicroorganisms found in the soil and onplant matter; inoculation occurrs as a resultof traumatic implantation of thorns, splin-ters, and other plant matter; Pseudallesche-ria boydii most common etiologic agent ofeumycetoma in the United States; Madurellamycetomatis most common causativeorganism worldwide

Clinical manifestationDisease presents as a painless swelling orthickening of the skin and subcutaneoustissue, usually over the distal lower extrem-ity; with progression over months or years;lesion enlarges and eventually becomestumorous; overlying skin sometimessmooth, dyspigmented, or shiny; abscessesand sinus tracts develops and sometimescontains a serosanguinous or seropurulentdischarge, with white-to-yellow or blackgranules; predisposing factors: walkingbarefoot, agricultural work; poor personalhygiene; poor nutrition, and wounds orskin infections

Differential diagnosisTuberculosis; leprosy; leishmaniasis; squa-mous cell carcinoma; yaws; syphilis; actino-mycetoma; botryomycosis; chromoblasto-mycosis; sporotrichosis; blastomycosis; coc-cidioidomycosis; elephantiasis

TherapyKetoconazole; itraconazole; surgical exci-sion

ReferencesRestrepo A (1994) Treatment of tropical mycoses.

Journal of the American Academy of Dermatol-ogy 31(3 Pt 2):S91–102

Eumycotic mycetoma

� Eumycetoma

European blastomycosis

� Cryptococcosis

Exanthem subitum

� Roseola


Exudative discoid and lichenoid dermatitis 231

E

Excoriated acne

� Acne excoriée

Exfoliative dermatitis

DefinitionEruption characterized by widespread ery-thema and scaling, often with pruritus,caused by multiple underlying conditionsincluding generalization of pre-existing dis-eases such as psoriasis and atopic dermati-tis; cutaneous T-cell lymphopma and reac-tions to medications

ReferencesRothe MJ, Bialy TL, Grant-Kels JM. (2000) Eryth-

roderma. Dermatologic Clinics 18(3):405–415

External otitis

� Otitis externa

Extramammary Paget’s disease

� Paget’s disease

Exudative discoid and lichenoid dermatitis

� Sulzberger-Garbe syndrome


F

Fabry disease

�


Fabry syndrome

�


Fabry-Anderson disease

�


Facial granuloma

�

Granuloma faciale

Facial ringworm

�

Tinea faciei

Factitial dermatitis

�

Dermatitis artefacta

Factitious urticaria

�

Dermatographism

Famciclovir

Trade name(s)

Famvir

Generic available

No

Drug class

Anti-viral

Mechanism of action

DNA polymerase inhibition

Dosage form

125 mg, 250 mg, 500 mg tablet

Dermatologic indications

See table

Common side effects

Gastrointestinal:

nausea, vomiting

Neurologic:

headache


None

Drug interactions

Probenecid


234 Familial atypical mole-melanoma syndrome


Hypersensitivity to drug class or compo-nent; elderly patients or those with renalfailure may need lower dose

References

Brown TJ, Vander Straten M, Tyring S (2001) An-tiviral agents. Dermatologic Clinics 19(1):23–34

Familial atypical mole-melanoma syndrome

�

Atypical mole

Familial baldness

�



Synonym(s)

Hailey-Hailey disease

;

familial benign pem-phigus

Definition

Inherited, intraepidermal, blistering dis-ease, affecting the neck, axillae, and groinarea

Pathogenesis

Autosomal dominant trait; overall defect inkeratinocyte adhesion, apparently second-ary to a primary defect in a calcium pumpprotein, ATP2C1; pump mutation inATP2C1, a gene localized on chromosome 3


Vesicles and erythematous plaques withoverlying crusts, usually occurring in thegenital area, the chest, neck, and axillaryregion; burning sensation and pruritusaccompanying the eruption; malodorousdrainage with secondary infection; factorsknown to exacerbate the disease: heat, fric-tion, and infection


Darier disease; impetigo; candidiasis; her-pes simplex virus infection; pemphigus vul-garis; pemphigus foliaceus; atopic dermati-tis; seborrheic dermatitis; extramammaryPaget’s disease

Therapy

Topical corticosteroids, mid potency; eryth-romycin, systemic; erythromycin, topical;

Famciclovir. Dermatologic indications and dosage


Herpes simplex virus infection, first epidose

250 mg PO 3 times daily for 7–10 days

Not established



Not established

Herpes simplex virus infection, recurrent episode

125 mg PO twice daily for 5 days Not established

Herpes zoster 500 mg PO 3 times daily for 7 days Not established

Varicella 500 mg PO 3 times daily for 7 days Not established


Faun-tail nevus 235

F

clindamycin, topical; local CO2 laser abla-tion; local dermabrasion

References

Gallagher TC (2000) Familial benign pemphigus. Dermatology Online Journal 6(1):7

Familial benign pemphigus

�


Familial hemorrhagic angiomatosis

�

Osler-Weber-Rendu syndrome

Familial Turner syndrome

�

Noonan’s syndrome

Familial white spotting

�

Piebaldism

Fanconi-Prader syndrome

�


Farcy

�

Glanders and melioidosis

Farmer’s neck

�

Actinic elastosis

Fatal cutaneointestinal syndrome

�


Fatal granulomatosis of childhood

�


Fatty tumor

�

Lipoma

Faun-tail nevus

�

Nevoid hypertrichosis

Familial benign chronic pemphigus.

Eroded plaques on the thighs


236 Favre-Chaix disease

Favre-Chaix disease

�

Acroangiodermatitis

Favre-Racouchot syndrome

Synonym(s)

Syndrome of Favre-Racouchot

;

nodularcutaneous elastoidosis with cysts andcomedones

;

senile comedones

;

solar come-dones

;

smoker's comedones

Definition

Disorder characterized by multiple openand closed comedones in actinically dam-aged skin

Pathogenesis

Unknown; develops in individuals with aheavy smoking history and chronic expo-sure to ultraviolet light


Multiple, bilaterally symmetrical, open andclosed comedones in the periorbital andtemporal areas; occasionally noted in thelateral neck, postauricular areas, and fore-arms; actinically damaged skin with yellow-ish discoloration, yellowish nodules, atro-phy, wrinkles, and furrows


Acne vulgaris; nevus comedonicus; colloidmilia; milia; trichoepithelioma; syringoma;sebaceous hyperplasia; xanthoma

Therapy

Comedone extraction; surgical excision;tretinoin

References

Sharkey MJ, Keller RA, Grabski WJ, McCollough ML (1992) Favre-Racouchot syndrome. A com-bined therapeutic approach. Archives of Der-matology 128(5):615–616

Febrile neutrophilic dermatosis

�


Female pattern baldness

�


Female pseudo Turner syndrome

�

Noonan’s syndrome

Fexofenadine

�

Antihistamines, second generation

Fibroepithelial polyp

�

Acrochordon

Fibroepithelioma of Pinkus

Synonym(s)

Pinkus tumor

;

premalignant fibroepithelialtumor

Definition

Premalignant epithelial tumor consisting ofcells resembling those of basal cell carci-noma


Fibrous papule 237

F

Pathogenesis

Unknown


Slowly enlarging, single or multiple, fleshy,pink or reddish, pedunculated papules witha broad base; occur exclusively on thetrunk, particularly over the lumbosacralarea


Seborrheic keratosis; acrochordon; nevussebaceus of Jadassohn; melanocytic nevus;amelanotic melanoma; neurofibroma

Therapy

Destruction by electrodesiccation andcurettage; elliptical excision

References

Stern JB, Haupt HM, Smith RR (1994) Fibroepi-thelioma of Pinkus. Eccrine duct spread of ba-sal cell carcinoma. American Journal of Dermatopathology 16(6):585–587

Fibroma

Synonym(s)

None

Definition

Benign fibrous tissue tumor

References

Weiss SW (1986) Proliferative fibroblastic lesions. From hyperplasia to neoplasia. American Jour-nal of Surgical Pathology 10 Suppl 1:14–25

Fibroma durum

�

Dermatofibroma

Fibroma simplex

�

Dermatofibroma

Fibromatosis

Synonym(s)

None

Definition

Benign fibrous tissue proliferation, inter-mediate in biological behavior betweenbenign fibroma and fibrosarcoma

References

Fisher C (1996) Fibromatosis and fibrosarcoma in infancy and childhood. European Journal of Cancer 32A(12):2094–2100

Fibrosarcoma of the skin

�

Dermatofibrosarcoma protuberans

Fibrous dysplasia of bone

�

McCune-Albright Syndrome

Fibrous dysplasia, polyostotic

�

McCune-Albright syndrome

Fibrous papule

�

Fibrous papule of nose/face


238 Fibrous papule of nose/face

Fibrous papule of nose/face

Synonym(s)

Fibrous papule

;

fibrous papule of the nose

Definition

Small facial papule with a characteristicfibrovascular component on histologicalexamination

Pathogenesis

Possibly remnant of a melanocytic nevus,or fibrohistiocytic lineage, or derived fromdermal dendrocytes


Solitary or multiple, dome-shaped, shiny,skin-colored or red-brown firm papules;usually located on the nose, but sometimesarising on the cheeks, chin, neck, lip, or theforehead


Nevus; basal cell carcinoma; trichoepitheli-oma; syringoma; milium; seborrheic kera-tosis; pyogenic granuloma; angiofibroma

Therapy

Surgical excision for cosmesis

References

Shea CR, Salob S, Reed JA, Lugo J, McNutt NS 91996) CD34-reactive fibrous papule of the nose. Journal of the American Academy of Der-matology 35(2 Pt 2):342–345

Fibrous sclerosis of the penis

�

Peyronie’s disease

Fibroxanthoma, atypical

�

Atypical fibroxanthoma

Fiessinger-Leroy syndrome

� Reiter syndrome

Fiessinger-Leroy-Reiter syndrome

� Reiter syndrome

Fifth disease

� Erythema infectiosum

Filariasis

Synonym(s)Lymphatic filariasis; bancroftian filariasis;brugian filariasis; onchocerciasis; Africanriver blindness; blinding filariasis; Robles’disease; loiasis; Loa loa

DefinitionDisease group caused by nematode para-sites of the order Filariidae, commonlycalled filariae

PathogenesisLymphatic filariasis caused by Wuchereriabancrofti, Brugia malayi, and Brugiatimori; cutaneous filariasis caused by Loaloa, Onchocerca volvulus, and Mansonellastreptocerca; Microfilariae in insect hostinoculated into vertebral host during feed-ing and completing their life cycle there

Clinical manifestationLymphatic filariasis – acute episode (ade-nolymphangitis): fever; inguinal or axillarylymphadenopathy; testicular and/or


Finger infection 239

F

inguinal pain; limb or genital swelling; skinexfoliation of the affected body part usu-ally occurring with resolution of an epi-sode; recurrent episodes of inflammationand lymphedema leading to lymphaticdamage with chronic swelling and elephan-tiasis of the legs, arms, scrotum, vulva, andbreastsOnchocerciasis: skin nodules over bonyprominences (i.e., onchocercomas); otherskin lesions including edema, pruritus, ery-thema, papules, altered pigmentation, andlichenificationLoiasis: large transient area of localizednonerythematous subcutaneous edema(Calabar swelling), most common aroundjoints

Differential diagnosisScrotal or testicular trauma; lymphoma;lymphogranuloma venereum; Milroy dis-ease; bacterial or fungal lymphadenitis; lep-rosy; non-filarial elephantiasis; hydrocele

TherapyLymphatic filariasis: diethylcarbamazine6 mg per kg PO per day for 12–21 days�

Oncocerciasis: ivermectin�

Loiasis: diethylcarbamazine 6 mg per kg POper day for 12–21 days�; albendazole

ReferencesTaylor MJ, Hoerauf A (2001) A new approach to

the treatment of filariasis. Current Opinion in Infectious Diseases 14(6):727–731

Finasteride

Trade name(s)Propecia

Generic availableNo

Drug classType II 5 α-reductase inhibitor

Mechanism of actionInhibition of 5-α reductase causes reducedconversion of testosterone to dihydrotesto-sterone in hair follicles



Common side effectsGenitourinary: decreased libido, impo-tence, decreased ejaculate volume




ReferencesMessenger AG (2000) Medical management of

male pattern hair loss. International Journal of Dermatology 39(8):585–586

Finger infection

� Paronychia

Finasteride. Dermatologic indications and dosage


Androgenetic alopecia in men

1 mg PO daily Not indicated


240 Fingernail infection

Fingernail infection

� Paronychia

Finkelstein's disease


Fire ant bite


Fish fancier's finger

� Mycobacterium marinum infection

Fish odor syndrome

Synonym(s)None

DefinitionMetabolic disorder causing the excretion ofa compound in sweat with the odor of rot-ting fish

PathogenesisAutosomal dominant trait; trimethylaminederived from carnitine or choline by theaction of bowel flora; defect in trimethyl-amine metabolism in the liver, resulting incompound with fish-like odor

Clinical manifestationFoul body odor; no skin lesions

Differential diagnosisBromhidrosis from other causes

TherapyDiet low in carnitine and choline (seafood,eggs, liver, peas, soy beans)�

ReferencesMitchell SC (1996) The fish-odor syndrome. Per-

spectives in Biology & Medicine 39(4):514–526

Fish skin ichthyosis


Fish tank granuloma


Fissured tongue

� Lingua plicata

Five-day fever

� Trench fever

Fixed drug eruption

Synonym(s)Fixed medication reaction; fixed eruption

DefinitionEruption occurring at the same site or siteseach time a given medication is adminis-tered


Florid cutaneous papillomatosis 241

F

PathogenesisProbably results from an immunologicallymediated inflammatory response to a givenmedication

Clinical manifestationDevelops 6–48 hours after administrationof the causative drug; common etiologicagents: aspirin, barbiturates, co-trimoxa-zole, phenolphthalein, sulfonamides, andtetracycline; pruritus and burning, occa-sionally accompanied by fever; starts as afew sharply demarcated, erythematousmacules that rapidly become erythematousplaques, usually on the lips, genitalia, andtrunk; lesions heal hyperpigmentation;recurrence in the same site with readminis-tration of the offending drug

Differential diagnosisContact dermatitis; herpes simplex virusinfection; chemical burn; bullous pemphig-oid; lupus erythematosus; psoriasis; por-phyria cutanea tarda; erythema multi-forme; erythema migrans; bullous diseaseof diabetes mellitus; post-inflammatoryhyperpigmentation; factitial disease

TherapyWithdrawal of offending drug�

ReferencesShiohara T, Mizukawa Y, Teraki Y (2002) Patho-

physiology of fixed drug eruption: the role of

skin-resident T cells. Current Opinion in Aller-gy & Clinical Immunology 2(4):317–323

Fixed eruption

� Fixed drug eruption

Fixed medication reaction

� Fixed drug eruption

Flegel disease

� Hyperkeratosis lenticularis perstans

Flegel’s disease

� Hyperkeratosis lenticularis perstans

Florid cutaneous papillomatosis

Synonym(s)None

DefinitionDisorder characterized by the rapid onsetof numerous warty papules on the trunkand extremities, often in association withmalignant acanthosis nigricans and/or signof Leser-Trelet and an internal malignancy

PathogenesisUnknown

Fixed drug eruption. Scaly, red plaque on the glans penis


242 Florid papillomatosis of nipple ducts

Clinical manifestationMultiple verrucous papules, beginning onthe extremities, particularly on the dorsa ofthe hands and wrists; papules sometimesinvolve the entire body, including the face;usually associated with signs of internalcancer and malignant acanthosis nigricansand sign of Leser-Trélat

Differential diagnosisWarts; acrokeratosis verruciformis of Hopf;seborrheic keratoses; epidermodysplasiaverruciformis

TherapyTreatment of underlying malignancy; topi-cal therapies not effective

ReferencesSchwartz RA (1993) Florid cutaneous papilloma-

tosis. Clinics in Dermatology 11(1):89–91

Florid papillomatosis of nipple ducts


Florid papillomatosis of the nipple


Fluconazole

Trade name(s)Diflucan

Generic availableNo

Drug classTri-azole antifungal agent

Mechanism of actionCell wall ergosterol inhibition by blocking14α-demethlyation of lanosterol

Dosage form50 mg, 100 mg, 150 mg, 200 mg tablet;50 mg per ml, 200 mg/ml suspension


Common side effectsCutaneous: skin eruptionGastrointestinal: nausea and vomiting,diarrhea, abdominal pain, dyspepsiaNeurologic: headache, dizziness, tastechanges

Serious side effectsCutaneous: angioedema, Stevens-JohnsonsyndromeGastrointestinal: hepatotoxicityHematologic: agranulocytosis, leukocytosisNeurologic: seizures

Drug interactionsAmitriptyline; barbiturates; buspirone; car-bamazepine; celecoxib; cyclosporine; dig-oxin; ergot alkaloids; glyburide/metformin;phenytoin; pimozide; protease inhibitors;quinidine; rifampin; statins; sulfonylureas;tacrolimus; theophyllines; warfarin

Contraindications/precautionsHypersensitivity to drug class or compo-nent; caution in patients with impairedrenal or hepatic function

ReferencesMeis JF, Verweij PE (2001) Current management

of fungal infections. Drugs 61(Suppl 1):13–25

Fluocinolone acetonide



Fluorouracil, topical 243

F

Fluoroderma

� Halogenoderma

Fluorouracil, topical

Trade name(s)Efudex; Fluoroplex; Carac; Adrucil

Generic availableNo

Drug classAntimetabolite

Mechanism of actionInhibition of DNA synthesis by blockingthymidylate synthetase

Dosage form0.5% cream; 1% cream; 5% cream; 1% solu-tion; 2% solution; 5% solution; 50 mg/mlsolution for intralesional injection

Fluconazole. Dermatologic indications and dosage


Angular cheilitis 150 mg PO once weekly for 2–4 weeks

3–6 mg per kg weekly for 2–4 weeks

Candidiasis associated with hyperimmuno-globulin E syndrome

150 mg PO daily for 1-3 weeks, depending on therapeutic response

3–6 mg per kg PO once weekly for 1-3 weeks, depending on therapeutic response

Candidiasis, cutaneous

150 mg PO once weekly for 2–4 weeks

3–6 mg per kg PO once weekly for 2–4 weeks

Candidiasis, oral (thrush)

150 mg PO once weekly for 2–4 weeks

3–6 mg per kg PO for 14–28 days

Candidiasis, vulvovaginal

150 mg PO for 1 dose 150 mg PO for 1 dose

Chronic paronychia 150 mg PO once weekly for 3–6 weeks


Leishmaniasis 200 mg PO daily for 6 weeks 3–6 mg per kg PO once daily for 6 weeks

Onychomycosis 150 mg PO once weekly for 3–6 months

3–6 mg per kg PO once weekly for 3–6 months

Sporotrichosis 200-400 mg PO daily until infection clears

3–6 mg per kg PO once daily until infection clears

Tinea corporis 150 mg PO once weekly for 2–4 weeks


Tinea cruris 150 mg PO once weekly for 2–4 weeks


Tinea pedis 150 mg PO once weekly for 2–4 weeks


Tinea versicolor 150 mg PO for 1–2 doses over 7-14 days

3–6 mg per kg PO for 1 dose; repeat in 7–14 days

White piedra 150 mg PO once weekly for 2–4 weeks



244 Fluoxetine


Common side effectsCutaneous: local pain, pruritus, burning,crusting, erosions, allergic contact dermati-tis, photosensitivity, hypopigmentation,hyperpigmentation



Contraindications/precautionsHypersensitivity to drug class or compo-nent; avoid excessive sun exposure while inuse

ReferencesJeffes EW 3rd., Tang EH (2000) Actinic keratosis.

Current treatment options. American Journal of Clinical Dermatology 1:167–179

Fluoxetine

� Selective serotonin reuptake inhibi-tor (SSRI)

Flurandrenolide


Fluticasone propionate


Fluorouracil, topical. Dermatologic indications and dosage


Actinic keratosis Apply 1–2 times daily for 3–6 weeks Apply 1–2 times daily for 3–6 weeks

Basal cell carcinoma Apply 1–2 times daily for 4–8 weeks Not applicable

Basal cell nevus syndrome;

Apply 1–2 times daily for 4–8 weeks Apply 1–2 times daily for 4–8 weeks

Bowenoid papulosis Apply 1–2 times daily for 4–8 weeks Not applicable

Carcinoma-in-situ (Bowen’s disease)

Apply 1–2 times daily for 4–8 weeks Not applicable

Disseminated superficial actinic porokeratosis

Apply twice daily for 4–8 weeks Apply twice daily for 4–8 weeks


Apply 2 times daily for 2–4 months Not applicable

Keratoacanthoma 50 mg per ml intralesional injection; repeat every 2 weeks for up to 5 times

Not applicable

Wart Apply twice daily for 6 weeks Apply twice daily for 6 weeks


Fogo selvagem 245

F

Focal acantholytic dyskeratosis

� Warty dyskeratoma

Focal dermal hypoplasia

Synonym(s)Goltz's syndrome, Goltz syndrome

DefinitionGenetic disorder characterized by distinc-tive skin abnormalities and a wide varietyof defects affecting the eyes, teeth, and skel-etal, urinary, gastrointestinal, cardiovascu-lar, and central nervous system

PathogenesisX-linked dominant, typically lethal inmales; mosaicism with random X-chromo-some inactivation (lyonization) likely; pro-found dysplasia of ectodermal, neuroecto-dermal, endodermal, and mesodermal ele-ments

Clinical manifestationPresent at birth, almost exclusively infemales; skin findings – symmetric, linear,reticulated, frequently tender, pink or red,thin skin; involved areas angular, atrophic,slightly raised, or depressed macules, withtelangiectasias; lesions follow the lines ofBlaschko; prominent involvement on thelower extremities, forearms, and cheeks;hernia-like outpouchings of fatty tissue;multiple raspberry-like papillomas arisingat junctions between the mucosa and theskin (i.e. perioral, perivulvar, perianal, peri-ocular junctions); apocrine nevi; multiplehydrocystomas; hypohidrosis; scalp andbody hair usually sparse; hair sometimesbrittle; dysmorphic facial features; otherabnormalities, include short stature, skele-tal abnormalities, mental retardation, dys-morphic ears, and ocular abnormalities

Differential diagnosisAicardi syndrome; incontinentia pigmenti;proteus syndrome; MIDAS syndrome; apla-sia cutis congenita; Adams-Oliver syn-drome

TherapyFlashlamp-pumped pulse dye laser for tel-angiectatic and erythematous skin lesions

ReferencesHardman CM, Garioch JJ, Eady RA, Fry L (1998)

Focal dermal hypoplasia: report of a case with cutaneous and skeletal manifestations. Clinical & Experimental Dermatology 23(6):281–285

Focal facial dysplasia

� Brauer’s syndrome

Focal septal panniculitis


Fogo selvagem

Synonym(s)Endemic pemphigus foliaceus; Brazilianpemphigus; Brazilian pemphigus foliaceus;South American pemphigus

DefinitionVariant of pemphigus foliaceus found incertain areas of Central America, SouthAmerica, and Tunisia

PathogenesisAutoimmune disease, with autoantibodiesspecific for desmoglein, which results inacantholysis and linical blistering; black flymay be vector of spread


246 Follicular mucinosis

Clinical manifestationSuperficial vesicles that rupture easily andleave erosions; positive Nikolsky sign; occa-sional total body erythroderma; no mucousmembrane involvement

Differential diagnosisImpetigo; lupus erythematosus; pemphigusvulgaris; seborrheic dermatitis; atopic der-matitis; subcorneal pustular dermatosis;epidermolysis bullosa; glucagonoma syn-drome; erythema multiformeOther causes of erythroderma: drug reac-tion; cutaneous T-cell lymphoma; psoria-sis; pityriasis rubra pilaris; contact dermati-tis

TherapySevere disease: prednisone; steroid sparingagents: azathioprine; cyclophosphamide;mycophenolate mofetilMild-to-moderate disease: high potencytopical corticosteroids

ReferencesSampaio SA, Rivitti EA, Aoki V, Diaz LA (1994)

Brazilian pemphigus foliaceus, endemic pem-phigus foliaceus, or fogo selvagem (wild fire). Dermatologic Clinics 12(4):765–776

Follicular mucinosis

Synonym(s)Alopecia mucinosa

DefinitionDegenerative mucinosis of pilosebaceousunits

PathogenesisPrimary idiopathic form; secondary formassociated with benign diseases such aslupus erythematosus, lichen simplex chron-icus, and angiolymphoid hyperplasia, andwith malignant diseases such as mycosisfungoides, Kaposi's sarcoma, and Hodg-kin's disease

Clinical manifestationPruritic, pink to yellow-white, follicularpapules and plaques; may be solitary ormultiple; face and scalp most common sites;non-scarring alopecia

Differential diagnosisAlopecia areata; telogen effluvium; andro-genetic alopecia; keratosis pilaris; lichenspinulosus; lichen planopilaris

TherapyHigh potency topical corticosteroid; triam-cinolone 3–5 mg per ml intralesional

ReferencesTruhan AP, Roenigk HH Jr (1986) The cutaneous

mucinoses. Journal of the American Academy of Dermatology 14(1):1–18

Folliculitis

Synonym(s)None

DefinitionInflammation of the hair follicles caused byinfection or physical or chemical irritation

PathogenesisInflammatory process caused by obstruc-tion or disruption of individual hair folli-cles and the associated pilosebaceous units

Clinical manifestationPrimary lesion a perifollicular papule orpustule; often appears as grid-like patternof multiple red papules and/or pustules onhair-bearing areas, such as the face, scalp,thighs, axilla, and inguinal area; predispos-ing factors: friction; perspiration; occlu-sion; shaving; hyperhidrosis; diabetes melli-tus or immunologic disorders; Staphylococ-cal nasal carriage; skin injuries; abrasions;surgical wounds; draining abscesses; skinocclusion for topical corticosteroid therapy


Folliculitis, eosinophilic pustular 247

F

Clinical variants:• Hot tub folliculitis: caused by psedo-monas organisms in tub or pool water; pru-ritic, edematous, erythematous papules orpustules in areas of skin occluded by abathing suit• Pityrosporum folliculitis: pruritic acne-form papules on the upper back, chest,upper arms, neck, chin, and sides of theface; caused by yeast forms of Pityrospo-rum ovale• Fungal folliculitis: caused by candidalspecies and dermatophytes; principle etio-logic agents: Trichophyton verrucosum andTrichophyton mentagrophytes; affects thecoarse hairs in the mustache and beard areain men• Staphylococcal folliculitis: follicular-based red papules and pustules, caused byStaphylococcal aureus

Differential diagnosisErythema toxicum; miliaria; insect bitereaction; scabies; acne; rosacea; perioraldermatitis; keratosis pilaris; acquired perfo-rating disease; pemphigus foliaceus; Darierdisease; Hailey-Hailey disease; pseudofol-liculitis barbae

TherapyTreatment of infection as per cultureresults; removal of inciting factors, such astight-fitting clothing; anti-inflammatorytherapy: tetracycline; doxycycline; minocy-cline; dapsone

ReferencesSadick NS (1997) Current aspects of bacterial in-

fections of the skin. Dermatologic Clinics 15(2):341–349

Folliculitis barbae traumatica

� Pseudofolliculitis barbae

Folliculitis decalvans

Synonym(s)None

DefinitionFinal common pathway of various types ofchronic folliculitis, producing progressivescarring

PathogenesisInflammatory process caused by obstruc-tion or disruption of individual hair folli-cles and the associated pilosebaceous units,leading to scarring alopecia because ofdestruction of the follicular units; role ofstaphylococcal follicular infection uncer-tain

Clinical manifestationOccurs in women after age 30 and in menfrom adolescence onward; bogginess orinduration of affected areas of the scalp orother hair-bearing sites; successive crops ofpustules; late finding of scarring alopecia

Differential diagnosisDissecting folliculitis; lupus erythemato-sus; lichen planopilaris; kerion; pseudope-lade of Brocq; follicular degeneration syn-drome; pemphigus vulgaris; pemphigusfoliaceus; Darier disease; Hailey-Hailey dis-ease; pseudofolliculitis barbae

TherapyTreatment of infection as per culture results

ReferencesBrooke RC, Griffiths CE (2001) Folliculitis decal-

vans. Clinical & Experimental Dermatology 26(1):120–122

Folliculitis, eosinophilic pustular



248 Folliculitis keloidalis

Folliculitis keloidalis

� Acne keloidalis

Folliculitis keloidalis nuchae

� Acne keloidalis

Folliculitis, perforating


Folliculitis, pityrosporom

� Folliculitis

Folliculitis ulerythema reticulata

� Ulerythema ophryogenes

Folliculoma

� Trichofolliculoma

Fong disease

� Nail-patella syndrome

Fordyce angiokeratoma


Fordyce’s disease

Synonym(s)Fordyce’s spots; Audry’s glands; pseudo-colloid lip mucous membrane sebacousmilia; pseudocolloid of the buccal mucosa;pseudocolloid of the lips; mucosal seba-ceous cysts

DefinitionDevelopmental anomaly characterized byenlarged ectopic sebacous glands on themucosa of the mouth and genitals

PathogenesisUnknown

Clinical manifestationAsymptomatic, 1–2 mm yellow papules onthe mucosal surfaces of the mouth and gen-italia; papules sometimes coalesce intoplaques

Differential diagnosisWarts; lichen planus; white sponge nevus


ReferencesMassmanian A, Sorni Valls G, Vera Sempere FJ

(1995) Fordyce spots on the glans penis. British Journal of Dermatology 133(3):498–500

Fordyce’s spots



Fox-Fordyce disease 249

F

Foreign body granuloma

Synonym(s)Foreign body reaction

DefinitionInflammatory response with granulomaformation as a reaction to exogenous mate-rial, usually an inanimate object

PathogenesisReaction to inert foreign materials too largeto be ingested by either neutrophils or mac-rophages

Clinical manifestationFirm, red papule or nodule, clearly distin-guishable from the surrounding normal tis-sue

Differential diagnosisSarcoidosis; granuloma annulare; granu-loma faciale; kerion; epidermoid cyst; zirco-nium or beryllium granuloma; Wegener’sgranulomatosis; lymphoma; tuberculosis;leprosy; lymphogranuloma venereum; spo-rotrichosis

TherapySurgical removal of foreign body�

ReferencesYoshitatsu S, Takagi T (2000) A case of giant pen-

cil-core granuloma. Journal of Dermatology 27(5):329–332

Foreign body reaction

� Foreign body granuloma

Fort Bragg fever

� Leptospirosis

Fox impetigo

� Impetigo

Fox-Fordyce disease

Synonym(s)Fox-Fordyce syndrome; apocrine miliaria

DefinitionChronic, pruritic, papular eruption localizeto areas where apocrine glands are found,such as the axilla

PathogenesisUnknown; keratin plug in the hair follicleinfundibulum obstructs the apocrineacrosyringium and produces apocrineanhidrosis; extravasation of sweat andinflammation possible causes of the intenseitching

Clinical manifestationPruritic, flesh-colored-to-reddish, smooth,dome-shaped, discrete, follicular or perifol-licular papules, most commonly in theaxilla, but sometimes also affecting theperiareolar, inframammary, and pubicareas; appear under conditions of heat,humidity, and friction; anhidrosis in theaffected area; more common in women

Differential diagnosisFolliculitis; pseudofolliculitis of the axilla;miliaria; milia; follicular hamartomas; hid-radenitis suppurativa

TherapyTretinoin; surgical excision of the axilla;liposuction-assisted curettage

ReferencesChae KM, Marschall MA, Marschall SF (2002) Ax-

illary Fox-Fordyce disease treated with liposuc-tion-assisted curettage. Archives of Dermatology 138(4):452–454


250 Fox-Fordyce syndrome

Fox-Fordyce syndrome

� Fox-Fordyce disease

Foxhole foot

� Immersion foot

Frambesia tropica

� Yaws

Francois dyscephaly syndrome

� Hallermann-Streiff syndrome

Freckles

� Ephelides

Freezing of tissue

� Frostbite

Frey’s syndrome

� Auriculotemporal syndrome� Gustatory sweating

Frostbite

Synonym(s)Freezing of tissue

DefinitionDamage to the skin and underlying tissuescaused by extreme cold

PathogenesisExtreme cold temperatures cause ice crys-tals to form in and around cells; red bloodcells and platelets congeal, causing clots andischemic damage; with skin warming, dam-age also caused by reperfusion

Clinical manifestationFrostnip: mildest and completely reversibleform of cold injury; blanching and numb-ness of the exposed areaSuperficial frostbite: early signs and symp-toms: sticking or pricking sensation, fol-lowed by development of pale, waxy skin;involved area becomes anesthetic; deepertissues remain softDeep frostbite: same signs and symptomsas superficial variant, but deep tissuesbecome hard and solid, and mottled bluish-gray discoloration develops; after warming,skin turns erythematous, edematous, withthrobbing pain; within 6 hours, bullaeappear, sometimes filled with clear fluid orwith blood; wet or dry gangrene some-times occurs after severe tissue injury

Differential diagnosisChilblains; Raynaud phenomenon; subcuta-neous fat necrosis; acrocyanosis; trenchfoot

TherapyRapid rewarming in warm water bath at37°-44° but avoidance of rewarming if dan-ger of refreezing is present�; aloe vera gelapplied 4 times daily; avoidance of thawingof frostbitten area if it cannot be keptthawed; no direct dry heat, such as a radia-tor, campfire, heating pad, or hair dryer, tothaw the frostbitten areas; no rubbing or


Furuncle 251

F

massaging of affected area; no disturbanceof blisters on frostbitten skin; avoidance ofsmoking or drinking alcoholic beverageswhile recovering�

ReferencesMurphy JV, Banwell PE, Roberts AH, McGrouther

DA (2000) Frostbite: pathogenesis and treat-ment. Journal of Trauma-Injury Infection & Critical Care 48(1):171–178000

Frostnip

� Frostbite

Fuchs’ syndrome III


Fujimoto’s disease

� Kikuchi’s syndrome

Fuller-Albright syndrome


Fungal mycetoma

� Eumycetoma

Fungal nail infection

� Onychomycosis

Furrowed tongue

� Lingua plicata

Furuncle

Synonym(s)Boil; carbuncle (aggregation of severalfuruncles); abscess; furunculosis (multipleor recurrent furuncles)

DefinitionAcute infection of the hair follicle and peri-follicular tissue, usually caused by a Staph-lococcal pathogen

PathogenesisS. aureus usual infective organism; host fac-tors: follicular abnormality; maceration;ingrown hair; friction; minor skin trauma;colonization in the nares; diabetes mellitus;immunosuppression; poor nutrition orhygiene; exposure to harsh irritants; car-buncles with predisposition to thicker areasof skin, such as nape of neck and thigh

Clinical manifestationOccurs only in hair-bearing areas; mostcommon on neck, face, axillae, buttocks,thighs; presents as red, painful papule ornodule, enlarging over a few days; sponta-neous rupture yields pus and necroticdebris; resolution with post-inflammatoryhyperpigmentation

Differential diagnosisHidradenitis suppurativa; folliculitis; acne;inflamed epidermoid cyst; myiasis; foreignbody reaction; factitial disease

TherapyMedical therapy: dicloxacillin; cephalexin;azithromycin.Surgical therapy: incision and drainage iffluctuance.


252 Furunculosis

General therapy: warm compresses applied3–4 times per day

ReferencesStulberg DL, Penrod MA, Blatny RA (2002) Com-

mon bacterial skin infections. American Fami-ly Physician 66(1):119–124

Furunculosis

� Furuncle


G

Gamasid rickettsiosis

�

Rickettsialpox

Gangrene

Synonym(s)

Mortification

Definition

Term used to describe the decay or death ofan organ or tissue caused by a lack of bloodsupply; a complication of infectious orinflammatory processes, injury, or degener-ative changes associated with chronic dis-eases

References

Cha JY, Releford BJ Jr, Marcarelli P (1994) Necro-tizing fasciitis: a classification of necrotizing soft tissue infections. Journal of Foot & Ankle Surgery 33(2):148–155

Gardner syndrome

Synonym(s)

Gardner’s syndrome; familial adenomatouspolyposis; familial polyposis of the colon

Definition

Disorder characterized by gastrointestinalpolyps, multiple osteomas, and skin andsoft tissue tumors

Pathogenesis

Autosomal dominant trait; mutations in theadenomatous polyposis coli gene on chro-mosome 5q21-22, which is a tumor suppres-sor gene


Multiple epidermoid cysts; desmoidtumors; fibrous tumors; osteomas, often onthe maxilla or mandible; congenital hyper-trophy of retinal pigment epithelium; mis-cellaneous findings: tyroid carcinoma;adrenal adenoma; urinary bladder carci-noma; hepatoblastoma


Peutz-Jeghers syndrome; Cowden disease;juvenile polyposis syndrome

Therapy

Early prophylactic colectomy

�

; geneticcounseling; surgical excision of cysts anddesmoids only for symptomatic relief

References

Tsao H (2000) Update on familial cancer syn-dromes and the skin. Journal of the American Academy of Dermatology 42(6):939-969


254 Garlic glove fibroma

Garlic glove fibroma

�

Acquired digital fibrokeratoma

Gas gangrene

Synonym(s)

Clostridial myonecrosis

Definition

Infection of muscle tissue by toxin-produc-ing clostridia organisms

Pathogenesis

Anaerobic, gram-positive, spore-formingbacillus of the genus

Clostridium

, of which

C perfringens

is the most common species;organism produces multiple exotoxins inju-rious to tissue


Posttraumatic gas gangrene: recent seriousinjury to the skin or soft tissues or openfracturesPostoperative gas gangrene: history ofrecent surgery of the gastrointestinal tractor the biliary tractOccult malignancy-associated spontaneousgas gangrene: no obvious preceding event;gas gangrene presents with sudden onset ofpain, with low-grade fever and apatheticmental status; local swelling and serosan-guineous exudate appear soon after onset ofpain; skin turns to a bronze color, thenprogresses to a blue-black color with hem-orrhagic bullae; entire region sometimesbecomes markedly edematous withinhours; wound may be nonodorous or have asweet mousy odor; crepitus following gasproduction; pain and tenderness to palpa-tion disproportionate to wound appear-ance; late signs: hypotension, renal failure,and a paradoxical heightening of mentalacuity


Cellulitis; necrotizing fasciitis; abortion;bacterial sepsis; abdominal abscess

Therapy

Combination of penicillin G and intrave-nous clindamycin

�

; combination of clin-damycin and metronidazole in penicillin-allergic patients; fasciotomy for compart-ment syndrome

�

; surgical debridement ofnecrotic tissue; hyperbaric oxygen

References

Chapnick EK, Abter EI (1996) Necrotizing soft-tissue infections. Infectious Disease Clinics of North America 10(4):835–855

Gastrointestinal polyposis syndrome, generalized, associated with hyperpigmentation, alopecia, and nail atrophy

�

Cronkhite-Canada syndrome

Gaucher’s disease

Synonym(s)

Glucosyl cerebroside lipidosis

;

glucosyl-ceramide lipidosis

Definition

Group of diseases resulting from an inbornerror of glycosphingolipid metabolismcaused by the deficient activity of the lyso-somal hydrolase, acid beta-glucosidase, andresulting in progressive accumulation ofundegraded glycolipid substrates, particu-larly glucosylceramide, in the bone mar-row, liver, and spleen

Pathogenesis

Autosomal recessive disorder; inborn errorof glycosphingolipid metabolism caused by


Gianotti-Crosti syndrome 255

G

the deficient activity of the lysosomalhydrolase, acid beta-glucosidase


Presenting symptom in all types: excessivefatigue associated with a hypochromic ane-mia and splenomegalyType 1 (adult nonneuronopathic form):onset of the manifestations from earlychildhood to late adulthood; generalizedyellowish bronze hyperpigmentation;bleeding, secondary to thrombocytopenia,manifested as epistaxis and ecchymoses;sequlae of monoclonal gammopathy ormultiple myelomaType 2 (infantile or acute neuronopathictype): collodion-type skin changes or ich-thyosis; hepatosplenomegaly; rapid neuro-logic deterioration, leading to death withinthe first year of lifeType 3 (juvenile, Norrbotten, or subacuteneuronopathic form): neurologic signs suchas deficits in eye movements, cerebellarabnormalities, tonic-clonic seizures, ormyoclonus; hypersplenism and skeletalchanges similar to those in the chronic non-neuronopathic form


Addison’s disease; phytophotodermatitis;traumatic ecchymosis

Therapy

Human placental and recombinant glu-cocerebrosidase; bone marow transplanta-tion

References

Schiffmann R, Brady RO (2002) New prospects for the treatment of lysosomal storage diseases. Drugs 62(5):733–742

Generalized lentiginosis

�

LEOPARD syndrome

Generalized lipodystrophy

�


Genetic hemochromatosis

�

Hemochromatosis

Genital wart

�

Condyloma acuminatum

Geographic tongue

�

Benign migratory glossitis

German measles

�

Rubella

Gianotti-Crosti syndrome

Synonym(s)

Papular acrodermatitis of childhood

;

papulovesicular acrolocated syndrome

;

acropapulo-vesicular syndrome

;

infantilepapular acrodermatitis

;

infantile lichenoidacrodermatitis

;

erythemato-papulous acro-dermatitis

;

erythemato-vesiculo-papulouseruptive syndrome

;

acrodermatitis papu-losa eruptiva infantilis

;

papular infantileacrodermatitis

;

acrodermatitis papulosa


256 Giant cell arteritis

infantum

;

infantile eruptive papulous der-matitis

Definition

Self-limited, childhood exanthem occur-ring in characteristic distribution and asso-ciated with multiple infectious agents

Pathogenesis

Associated with mostly viral agents, includ-ing hepatitis B, Epstein-Barr virus (EBV),respiratory syncytial virus (RSV), coxsack-ievirus and other enteroviruses, parainflu-enza virus, parvovirus B19, poxvirus,cytomegalovirus (CMV), humanherpesvirus 6 (HHV-6); some occurrencesfollow immunization with measles-mumps-rubella, poliovirus, and influenza virus vac-cines


Pale, pink-to-flesh-colored papules local-ized symmetrically over the extremities, thebuttocks, and the face; papules sometimeshave a smooth-topped, polished, orlichenoid appearance; occasional pruritus;occasional lymphadenopathy and mild con-stitutional symptoms, such as low-gradefever and malaise; complete resolution afterat least 2 months


Pityriasis rosea; pityriasis lichenoides;atopic dermatitis; lichen planus; lichennitidus; drug eruption; Langerhans cell his-tiocytosis; flat warts; polymorphous lighteruption; sarcoidosis; granuloma annulare;scabies

Therapy

None

References

Nelson JS, Stone MS (2000) Update on selected vi-ral exanthems. Current Opinion in Pediatrics 12(4):359–364

Giant cell arteritis

�

Temporal arteritis

Giant cell reticulohistiocytosis

�

Multicentric reticulohistiocytosis

Giant condyloma of Buschke and Löwenstein

Synonym(s)

Giant condylomata acuminata of Buschkeand Löwenstein

;

anogenital verrucous car-cinoma

;

Buschke-Löwenstein tumor

;

giantmalignant condyloma

Definition

Slow-growing, locally destructive, verru-cous carcinoma, typically appearing on thepenis but sometimes occurring elsewhere inthe anogenital region

Pathogenesis

Unclear; possibly a human papilloma virus-induced neoplasm; other possible etiologicfactors: chronic phimosis and poor penilehygiene


Presents on the prepuce as a keratoticplaque, slowly expanding into a cauliflower-like mass; sometimes ulcerate or forms apenile horn; associated with a foul odor;expansion to the corpus cavernosum andurethra may occur with subsequent fistula-tion; regional lymphadenopathy common,primarily due to secondary infection


Glanders and melioidosis 257

G


Condyloma acuminata; squamous cell car-cinoma

Therapy

Surgical excision

�

; interferon

References

Kanik AB, Lee J, Wax F, Bhawan J (1997) Penile verrucous carcinoma in a 37-year-old circum-cised man. Journal of the American Academy of Dermatology 37(2 Pt 2):329–331

Giant condylomata acuminata of Buschke and Löwenstein

�


Giant follicle

�

Dilated pore

Giant hemangioma syndrome

�

Kasabach-Merritt syndrome

Giant malignant condyloma

�


Gingivitis, desquamative

�

Desquamative gingivitis


Synonym(s)

Farcy

;

morve

;

malleus

(glanders);

Whitmore disease

(melioidosis)

Definition

Related diseases produced by bacteria of the

Burkholderia

species, which are gram-nega-tive rods

Pathogenesis

Causative agent of Glanders: Burkholderiamallei; primarily a disease of animals suchas horses, mules, and donkeys; once in thehost, synthesis and release of certain toxinsoccur; melioidosis: caused by the bacte-rium Burkholderia pseudomallei; organismdistributed widely in the soil and water ofthe tropics and spread to humans throughdirect contact with a contaminated source


Similar clinical syndrome in both diseases.Localized form: bacteria enter the skinthrough a laceration or abrasion; localinfection with ulceration and regional lym-phadenopathy; incubation period 1–5 days;bacteria that enter the host throughmucous membranes sometimes causeincreased mucus production in the affectedareasPulmonary form: occurs when bacteria areaerosolized and enter respiratory tract viainhalation or hematogenous spread; withinhalational melioidosis, cutaneousabscesses may develop; septicemia: whenbacteria disseminated in the bloodstreamin glanders, usually fatal within 7–10 daysChronic form: multiple abscesses affectingthe liver, spleen, skin, or muscles


Anthrax; plague; tuberculosis; atypicalmycobacterial infection; brucellosis; NorthAmerican blastomycosis; coccidioidomyco-sis; nocardia infection


258 Glomangioma

Therapy

Amoxicillin; tetracycline

References

Rosenbloom M, Leikin JB, Vogel SN, Chaudry ZA (2002) Biological and chemical agents: a brief synopsis. American Journal of Therapeutics 9(1):5–14

Glomangioma

�

Glomus tumor

Glomus tumor

Synonym(s)

Glomangioma

Definition

Benign neoplasm of modified smooth mus-cle cells (glomus cells)

Pathogenesis

Unknown cause for solitary lesion; multi-ple glomus tumors, especially those of thedisseminated form, inherited as autosomal-dominant trait with incomplete pene-trance; tumors arise from the arterial por-tion of the glomus body


Solitary glomus tumor: paroxysmal pain,which can be severe and exacerbated bypressure or temperature changes, especiallycold; blanchable blue or purple papule,located most commonly in acral areas,especially subungual areas of fingers andtoesMultiple glomus tumors: pain relativelyuncommon

•

Regional variant: blue-to-purple, com-pressible papules or nodules that aregrouped and limited to a specific area, mostcommonly an extremity

•

Disseminated variant: multiple lesionsdistributed over the body with no specific

grouping; congenital plaquelike glomustumors: grouped papules coalescing intoindurated plaques or clusters of discretenodules


Angioleiomyoma; angiolipoma; arteriov-enous malformation; blue nevus; hemangi-oma; melanoma; spiradenoma; tufted angi-oma; Kaposi’s sarcoma; blue rubber blebnevus; neurilemmoma

Therapy

Solitary glomus tumor: surgical excision

�

;multiple glomus tumors: surgical removalfor cosmetic reasons only

References

Alam M, Scher RK (1999) Current topics in nail surgery. Journal of Cutaneous Medicine & Sur-gery 3(6):324–335

Parsons ME, Russo G, Fucich L, Millikan LE, Kim R (1997) Multiple glomus tumors. International Journal of Dermatology 36(12):894-900

Glossodynia

Definition

Painful sensation in the tongue

References

Marbach JJ (1999) Medically unexplained chronic orofacial pain. Temporomandibular pain and dysfunction syndrome, orofacial phantom pain, burning mouth syndrome, and trigemi-nal neuralgia. Medical Clinics of North Ameri-ca 83(3):691–710

Glucagonoma syndrome

Synonym(s)Necrolytic migratory erythema

DefinitionGlucagon-secreting tumor associated withhyperglucagonemia, necrolytic migratory


Goltz-Gorlin syndrome 259

G

erythema, and diabetes mellitus; hypoami-noacidemia; cheilosis; normochromic, nor-mocytic anemia; venous thrombosis;weight loss; neuropsychiatric signs andsymptoms; pseudoglucagonoma syndrome:necrolytic migratory erythema without aglucogon-secreting tumor, but with anotherunderlying cause such as cirrhosis, celiacsprue, or pancreatitis

PathogenesisUnclear relation between glucagonoma andskin findings; levels of glucagon not wellcorrelated with the episodic course of theskin manifestations; possible role of rela-tive zinc deficiency; theories of causation:related to glucagon-induced hypoalbumine-mia; zinc-dependent delta-6 desaturation oflinoleic acid; poor hepatic breakdown ofglucagon contributing to an excessive pros-taglandin-mediated inflammatory response

Clinical manifestationPresents with nonspecific complaints, suchas weight loss, diabetes mellitus, diarrhea,and stomatitis; necrolytic migratory ery-thema: found anywhere on the body, butmost common in the perineum, buttocks,groin, lower abdomen, and lower extremi-ties; eruption starts as a pruritic or painful,erythematous patch that blisters centrally,erodes, crusts over, and heals with hyper-pigmentation; annular lesions with conflu-ence into plaques; confluence in severelyaffected areas; associated mucocutaneousfindings, including atrophic glossitis,cheilosis, dystrophic nails, and buccalmucosal inflammation

Differential diagnosisAcrodermatitis enteropathica; candidiasis;paraneoplastic pemphigus; Hailey-Haileydisease; Darier disease; pellagra; kwash-iorkor

TherapySurgical resection of the tumor, if local-ized�; in the absence of tumor, treat under-lying cause�

ReferencesChastain MA (2001) The glucagonoma syndrome:

a review of its features and discussion of new perspectives. American Journal of the Medical Sciences 321(5):306–320

Glucosyl cerebroside lipidosis

� Gaucher’s disease

Glucosylceramide lipidosis

� Gaucher’s disease

Glycolic acid

� Alpha hydroxy acid

Glyderm plus


Goltz syndrome

� Focal dermal hypoplasia

Goltz-Gorlin syndrome



260 Goltz's syndrome

Goltz's syndrome


Gonadal dysgenesis

� Turner syndrome

Gonococcal dermatitis-arthritis syndrome

� Gonococcemia

Gonococcemia

Synonym(s)Gonococcal dermatitis-arthritis syndrome;disseminated gonococcal infection

DefinitionSexually transmitted disease caused by thebacterium Neisseria gonorrhoeae, whichspreads from the initial site of infection

through the bloodstream to other parts ofthe body

PathogenesisNeisseria gonorrhoeae transmitted throughvaginal, oral, and anal intercourse; infec-tion also transmitted by a woman to hernewborn during childbirth; disseminationoften occurs during menses

Clinical manifestationMore common in women, often withasymptomatic infection; disseminated dis-ease generally follows the primary genitalinfection by several days to 2 weeks; fever;myalgias; tenosynovitis; monoarticular sep-tic arthritis, affecting large, weight-bearingjoints; acral palpable purpuric papules andpustules, usually relatively few in number

Differential diagnosisMeningococcemia or other infectiouscauses of septic vasculitis; lupus erythema-tosus; cryoglobulinemia; Reiter syndrome;infective endocarditis

TherapyCeftriaxone 1 gm intramuscularly or intra-venously every 24 hours for 3 days or until24 hours after symptomatic improvement;complete 7-day course with ciprofloxacin500 mg PO twice daily or cefixime 400 mgPO twice daily or azithromycin 500 mg POper day�; concurrent therapy for presumedchlamydia with doxycycline 100 mg POtwice daily for 7 days



Gorlin syndrome


Gonococcemia. Violaceous papule on the toe


Graft versus host disease 261

G

Gorlin-Goltz syndrome


Gottron’s syndrome

� Acrogeria

Gougerot and Blum, lichenoid pigmented purpura

� Benign pigmented purpura

Gougerot-Carteaud papillomatosis


Gougerot-Houwer-Sjögren syndrome

� Sjögren syndrome

Gowers’ local panatrophy

� Panatrophy of Gowers

Gowers’ panatrophy

� Panatrophy of Gowers

Graft versus host disease

Synonym(s)

DefinitionImmunologic assault and its consequenceswhen immunologically competent cells areintroduced into an immunoincompetenthost

PathogenesisThree criteria for development – (1) graftcontaining immunologically competentcells, (2) host appearing foreign to the graft,(3) host incapable of reacting sufficientlyagainst the graft; recognition of epithelialtarget tissues as foreign by the immuno-competent cells, with subsequent inductionof an inflammatory response and eventualapoptotic death of the target tissue; reac-tion against the host's keratinocytes, result-ing in the clinical syndrome

Clinical manifestationIncidence higher in recipients of allogeneichematopoietic cells than in patients receiv-ing syngeneic or autologous hematopoietic

Graft versus host disease. Sclerotic, hyperpigmented and hypopigmented plaques on the upper trunk


262 Granular bacteriosis

cells; greatest incidence in patients in whombone marrow is used as the source ofhematopoietic cellsAcute graft versus host disease: observed10–30 days posttransplant; eruptions usu-ally begin as faint, tender, erythematousmacules, often centered around hair folli-cles; as disease progresses, macules some-times coalesce to form confluent plaques orpapules; subepidermal bullae may occurChronic graft versus host disease: evolvesfrom acute form in 70–90% of patients; riskincreases with the severity of acute reac-tion; violaceous lichenified papules, oftenon the ventral skin surfaces, very similar tothose of lichen planus; lacy white plaqueson the buccal mucosa; scattered scleroder-matous plaques; widespread disease result-ing in ulcerations, joint contractures, andesophageal dysmotility

Differential diagnosisAcute graft versus host disease: erythemamultiforme; drug eruption; Stevens-John-son syndrome/toxic epidermal necrolysis;eruption of lymphocyte recoveryChronic graft versus host disease: sclero-derma; lichen planus; lichenoid drug erup-tion; lupus erythematosus

TherapyAcute graft versus host disease: pred-nisone; extracorporeal photochemotherapyChronic graft versus host disease: photo-chemotherapy; methotrexate; extracorpor-eal photochemotherapy; hydroxychloro-quine; etretinate

ReferencesJacobsohn DA, Vogelsang GB (2002) Novel phar-

macotherapeutic approaches to prevention and treatment of GVHD. Drugs 62(6):879–889

Granular bacteriosis

� Botryomycosis

Granular cell myoblastoma


Granular cell neurofibroma


Granular cell neuroma


Granular cell schwannoma


Granular cell tumor

Synonym(s)Granular cell myoblastoma; granular cellschwannoma; granular cell neuroma;granular cell neurofibroma; Abrikossof’stumor

DefinitionAcquired tumor of neural crest origin, char-acterized by cells with eosinophilic cyto-plasmic granules

PathogenesisPossible tumor derivation from Schwanncells

Clinical manifestationDiscrete, asymptomatic, firm, flesh-colored nodule, located within or beneaththe dermis, occurring in the tongue, head,


Granuloma faciale 263

G

and neck region or dorsal aspect of theforearms

Differential diagnosisFibroma; squamous cell carcinoma; wart;dermatofibroma; neurofibroma; epider-moid cyst


ReferencesBecelli R, Perugini M, Gasparini G, Cassoni A, Fa-

biani F (2001) Abrikossoff 's tumor. Journal of Craniofacial Surgery 12(1):78–81

Granuloma, actinic


Granuloma annulare

Synonym(s)None

DefinitionInflammatory skin disease characterized byannular plaques consisting of small papules

PathogenesisMay involve immune mechanisms

Clinical manifestationLocalized variant: flesh-colored to dull redpapules, often in an annular arrangement,over distal extremities; often occur overdorsal surfaces of feet, hands and fingers,and the extensor aspects of arms and legsGeneralized variant: few to thousands offlesh-colored to dull red papules involvingmultiple body regions, often in symmetri-cal distribution; papules may coalesce intoannular or arcuate plaques; may have largered patches (vascular granuloma annulare)Subcutaneous variant: firm, nontender,flesh-colored-to-pinkish papules or nod-ules without overlying epidermal altera-tion, often over the lower extremity

Differential diagnosisErythema annulare centrifugum; tinea cor-poris; lichen planus; lupus erythematosus;insect bite reaction; sarcoidosis; Lyme dis-ease; necrobiosis lipoidica; rheumatoidnodules; acquired perforating disease;lichen myxedematosus; cutaneous T-celllymphoma; erythema multiforme

TherapyLocalized disease: intralesional triamci-nolone; corticosteroids, topical, superpo-tentGeneralized disease: photochemotherapy

ReferencesSmith MD, Downie JB, DiCostanzo D (1997)

Granuloma annulare. International Journal of Dermatology 36(5):326–333

Granuloma faciale

Synonym(s)Facial granuloma; granuloma faciale eosi-nophilicum, granuloma faciale with eosi-nophilia

Granuloma annulare. Annular red-brown plaques on the dorsal aspect of the hand


264 Granuloma faciale eosinophilicum

DefinitionBenign chronic skin disease of unknownorigin, characterized by single or multiplecutaneous nodules, usually occurring overthe face

PathogenesisSun exposure possible factor in develop-ment

Clinical manifestationSolitary or multiple, sharply marginated,red or violaceous papules or nodules; sur-face sometimes has telangiectasias and/orenlarged follicular orifices; usually occurson the face, but also on the upper extremi-ties or trunk

Differential diagnosisSarcoidosis, granuloma annulare; discoidlupus erythematosus; mycosis fungoides;fixed drug eruption; Jessner’s lymphocyticinfiltrate; lymphoma; leprosy; lupus vul-garis; foreign body granuloma

TherapyTriamcinolone 3–4 mg per ml intralesional;dapsone

ReferencesInanir I, Alvur Y (2001) Granuloma faciale with

extrafacial lesions. British Journal of Dermatol-ogy 145(2):360–362

Granuloma faciale eosinophilicum

� Granuloma faciale

Granuloma faciale with eosinophilia

� Granuloma faciale

Granuloma fissuratum

� Acanthoma fissuratum

Granuloma gluteale infantum

Synonym(s)Kaposi’s sarcoma-like granuloma;granuloma intertriginosum infantum;infantile vegetating halogenosis; vegetatingpotassium bromide toxic dermatitis;vegetating bromidism

DefinitionDisease characterized by oval, granuloma-tous nodules on the gluteal surfaces andgroin areas of infants

PathogenesisUnclear; unusual cutaneous response tolocal inflammation, maceration, and sec-ondary infection; contact occlusion proba-bly predisposing factor

Clinical manifestationSolitary or mulptiple, red-purple to red-brown, firm-to-hard, discrete dermal nod-ules with smooth or slightly lichenified sur-faces; aligned with the long axis parallel tothe skin folds; located on the gluteal sur-faces, in the groin area, upper thighs, lowerabdomen, or rarely the neck and face

Differential diagnosisLangerhans cell histiocytosis; candidiasis;contact dermatitis; lymphoma; mastocyto-sis; scabies; syphilis; juvenile xanthogranu-loma; pyogenic granuloma; sarcoma; for-eign body granuloma

TherapySpontaneous resolution; no therapy indi-cated


Granulomatosis disciformis chronica et progressiva 265

G

ReferencesBluestein J, Furner BB, Phillips D (1990) Granulo-

ma gluteale infantum: case report and review of the literature. Pediatric Dermatology 7(3):196–198

Granuloma inguinale

Synonym(s)Donovanosis

DefinitionSexually transmitted disease characterizedby genital lesions presenting as indolent,progressive ulcerations with a granuloma-tous appearance

PathogenesisInfection caused by a gram-negative pleo-morphic bacillus, Calymmatobacteriumgranulomatis; mode of transmission prima-rily through sexual contact; mildly conta-gious

Clinical manifestationOccurs on glans penis and scrotum in men,and labia minora, mons veneris, and four-chette in women; rare cervical involve-ment; soft, red papules or nodules arising atthe site of inoculation; lesions eventuallyulcerate and produce red, friable, granulo-matous plaques and nodules; ulcers withclean, friable bases and distinct, raised,rolled margins; autoinoculation results inlesions on adjacent skin; occasional hyper-trophic or verrucous plaques, with forma-tion of large, vegetating masses resemblinggenital warts; swelling of the external geni-talia in later-stage lesions

Differential diagnosisSyphilis; lymphogranuloma venereum;chronic herpes simplex virus infection;squamous cell carcinoma; lichen sclerosus

TherapyTrimethoprim/sulfamethoxazole; doxycy-cline


overview of sexually transmitted diseases. Part 1. Journal of the American Academy of Derma-tology 41(4):511–532

Granuloma intertriginosum infantum

� Granuloma gluteale infantum

Granuloma pyogenicum

� Pyogenic granuloma

Granuloma telangiectaticum


Granuloma trichophyticum

� Majocchi granuloma

Granuloma tricofitico

� Majocchi granuloma

Granulomatosis disciformis chronica et progressiva



266 Granulomatosis, lymphomatoid

Granulomatosis, lymphomatoid


Granulomatosis, Miescher’s

� Miescher's granulomatosis

Granulomatous arteritis


Granulomatous cheilitis

� Cheilitis granulomatosa

Granulomatous disease of childhood


Granulomatous perioral dermatitis


Granulomatous rosacea

� Rosacea

Granulomatous vasculitis

� Wegener’s granulomatosis

Granulomatous vasculitis with asthma


Griscelli syndrome

Synonym(s)Partial albinism with immunodeficiency

DefinitionDisease characterized by partial pigmen-tary dilution with silvery gray hair, fre-quent infections, cellular immune defi-ciency, neurologic abnormalities, and fataloutcome from an uncontrolled T lym-phocyte and macrophage activation syn-drome

PathogenesisCaused by two genes: MYA5 and RAB27A;gene MYA5 produces severe neurologicalproblems; gene RAB27A causes acceleratedphase sometimes lethal within a shortperiod of time

Clinical manifestationSilvery blond hair; occasional subtle pig-mentary dilution of the skin and iris andhyperpigmentation in sun-exposed areas;accelerated phase of the disease with fever,jaundice, hepatosplenomegaly, lymphaden-opathy, pancytopenia and generalized lym-phohistiocytic infiltrates of various organsincluding the central nervous system; neu-rologic manifestations: hyperreflexia, sei-zures, signs of intracranial hypertension,regression of developmental milestones,


Griseofulvin 267

G

hypertonia, nystagmus, and ataxia; varietyof immunological abnormalities, restrictedto the patients with RAB27A defect

Differential diagnosisHematophagic lymphohistiocytosis; famil-ial lymphohistiocytosis; Chediak-Higashisyndrome; X-linked lymphoproliferativesyndrome

TherapyBone marrow transplantation�; chemother-apy for accelerated phase

ReferencesKlein C, Philippe N, Le Deist F, Fraitag S, Prost C,

Durandy A, Fischer A, Griscelli C (1994). Partial albinism with immunodeficiency (Griscelli syndrome). Journal of Pediatrics 125(6):886–895

Griseofulvin

Trade name(s)Fulvicin P/G; Gris-PEG; Grifulvin V


Drug classOral anti-fungal agent

Mechanism of actionInhibition of fungal cell wall synthesis

Dosage form125 mg, 165 mg, 250 mg, 330 mg tablet;125 mg per 5 ml suspension


Common side effectsCutaneous: photosensitivity, vascular reac-tionGastrointestinal: nausea, vomiting,diarrhea, flatulenceNeurologic: dizziness, paresthesias, confu-sion

Serious side effectsBone marrow: granulocytopeniaGastrointestinal: hepatotoxicity

Drug interactionsAmiodarone; barbiturates; carbamazepine;clarithromycin; oral contraceptives;cyclosporine; erythromycin; itraconazole;ketoconazole; protease inhibitors; tac-rolimus; warfarin

Griseofulvin. Dermatologic indications and dosage


Onychomycosis 500 mg PO twice daily for 6–12 months

5–10 mg per kg PO daily for 6–12 months

Tinea capitis 250–500 mg PO twice daily for 6–8 weeks

25 mg per kg PO daily for 6–8 weeks

Tinea corporis 250–500 mg PO twice daily for 2–6 weeks

5–10 mg per kg PO daily for 2–4 weeks

Tinea cruris 250–500 mg PO twice daily for 2–6 weeks

5–10 mg per kg PO daily for 2–4 weeks

Tinea faciei 250–500 mg PO twice daily for 2–6 weeks

25 mg per kg PO daily for 6–8 weeks


268 Groin dermatophytosis


Contraindications/precautionsHypersensitivity to drug class or compo-nent; acute intermittent porphyria; preg-nancy; caution in patients with penicillinallergy or impaired liver function

ReferencesBennett ML, Fleischer AB. Loveless JW, Feldman

SR (2000) Oral griseofulvin remains the treat-ment of choice for tinea capitis in children. Pediatric Dermatology 17(4):304–309

Groin dermatophytosis

� Tinea cruris

Grönblad-Strandberg syndrome

� Pseudoxanthoma elasticum

Groove sign

DefinitionEnlargement of the nodes above and belowthe inguinal ligament in patients with lym-phogranuloma venereum



Grover disease

� Transient acantholytic dermatosis

Grover’s disease

� Transient acantholytic dermatosis

Gumma

DefinitionSoft, tumor-like granulomatous growthcaused by syphilis, appearing during thelate stage, tertiary syphilis, most frequentlyin the liver but also occurring in the brain,testis, heart, skin, and bone

ReferencesQuinn P, Weisberg L (1997) Cerebral syphilitic

gumma. New England Journal of Medicine. 336(14):1027–1028

Günther’s disease

� Erythropoietic porphyria� Congenital erythropoietic porphyria

Gustatory hyperhidrosis


Gustatory sweating



Gym itch 269

G

Guttate parapsoriasis

� Pityriasis lichenoides� Small plaque parapsoriasis

Guttate psoriasis

� Psoriasis

Gym itch

� Tinea cruris


H

Haber’s syndrome

Synonym(s)

None

Definition

Rosacea-like eruption with keratoticpapules and pitted scars

Pathogenesis

Unknown; familial incidence


Permanent flushing of the cheeks, nose,forehead and chin, with erythema and tel-angiectasia; keratotic papules; atrophic,pitted papules; prominent follicles; come-dones


Rosacea; polymorphous light eruption; seb-orrheic dermatitis; lupus erythematosus;tinea faciei; Dowling Degos disease

Therapy

Light hyfrecation or cryotherapy of kera-totic papules; no effective therapy for ery-thema

References

McCormack CJ, Cowen P (1997) Haber's syn-drome. Australasian Journal of Dermatology 38(2):82–84

Hailey-Hailey disease

�


Hair follicle nevus

�

Trichofolliculoma

Hairy leukoplakia

Synonym(s)

Oral hairy leukoplakia

Definition

Oral infection caused by the Epstein-Barrvirus, appearing as white, mildly verrucouslesions on the lateral surfaces of the tongue

Pathogenesis

Caused by Epstein-Barr virus; unclearwhether a development following superin-fection with EBV or activation of latentinfection due to reduced immune surveil-lance


Asymptomatic, white plaque along the lat-eral tongue borders, with accentuation ofvertical folds; occasionally spreads to the


272 Hairy tongue

mouth floor, tonsillar pillars, ventraltongue, and pharynx; occurs almost exclu-sively in immunocompromised patients,particularly those infected with HIV


Wart; syphilis; premalignant leukoplakia(“smoker’s leukoplakia”); traumatic leuko-plakia; squamous cell carcinoma; candidia-sis; geographic tongue; lichen planus

Therapy

None

References

Itin PH, Lautenschlager S, Fluckiger R, Rufli T (1993) Oral manifestations in HIV-infected pa-tients: diagnosis and management. Journal of the American Academy of Dermatology 29(5 Pt 1):749–760

Hairy tongue

Synonym(s)

Black hairy tongue

;

lingua nigra

;

lingua vil-losa

;

lingua villosa nigra

Definition

Condition of defective desquamation of thefiliform papillae of the tongue that resultsin an irregular, discolored plaque, withelongation of filiform papillae and a lack ofnormal desquamation

Pathogenesis

Inadequate hygiene or microbial over-growth stimulates elongation of filiformpapillae; lack of mechanical stimulationand debridement


Elongation of the filiform papillae on thedorsal surface of the tongue, which retainpigments from food, beverages, andtobacco, resulting in brown, black or red-dish discoloration


Candidiasis; lichen planus; oral hairy leuko-plakia

Therapy

Mechanical removal of elongated papillaeby brushing the tongue with a toothbrushor using a tongue scraper; destruction byelectrodesiccation and curettage or CO2laser vaporization; tretinoin; acitretin

References

Sarti GM, Haddy RI, Schaffer D, Kihm J (1990) Black hairy tongue. American Family Physician 41(6):1751–1755

Halcinonide

�

Corticosteroids, topical, high potency

Half-and-half nails

Definition

Distal portion of the nail plate assuming areddish-brown color while more proximalportion remaining white; seen in patientswith renal disease and in many normal peo-ple

Hairy tongue.

Brown, hypertrophic plaque on the tongue


Halo nevus 273

H

References

Mazuryk HA, Brodkin RH (1991) Cutaneous clues to renal disease. Cutis 47(4):241–248

Hallermann-Streiff syndrome

Synonym(s)

Francois dyscephaly syndrome

;

Hallermann-Streiff-Francois syndrome

;

oculomandibulodyscephaly with hypotri-chosis

;

oculomandibulofacial syndrome

Definition

Genetic disorder characterized by malfor-mations of the skull and facial region,sparse hair, ocular abnormalities, dentaldefects, degenerative skin changes, andshort stature

Pathogenesis

Unknown


Skin findings: sparse hair; atrophy, particu-larly in the scalp and nasal regionsCraniofacial features: brachycephaly withfrontal and/or parietal bossing; small,underdeveloped lower jaw; narrow, highlyarched palate; thin, pinched, tapering noseOcular findings: congenital cataracts;microphthalmia; other ocular abnormali-tiesDental defects: presence of natal teeth;hypodontia or partial adontia malforma-tion; and/or improper alignment of teethSkeletal findings: short stature


Progeria; Werner’s syndrome

Therapy

None

References

Cohen MM Jr (1991) Hallermann-Streiff syn-drome: a review. American Journal of Medical Genetics 41(4):488–499

Hallermann-Streiff-Francois syndrome

�


Hallopeau, acrodermatitis continua

�

Acrodermatitis continua of Hallo-peau

Halo nevus

Synonym(s)

Sutton’s nevus

;

nevus of Sutton

;

leukoderma acquisita centrifugum

Definition

Benign skin lesion representing melano-cytic nevus in which an inflammatoryresponse produces zone of depigmentationsurrounding the lesion

Pathogenesis

Unclear; apparently an immunologic reac-tion against melanocyte; cells predomi-nantly T lymphocytes; precipitating causeand exact role of lymphocytes unknown


One or more, uniformly colored, evenlyshaped, round or oval pigmented papulesor macule, with regular peripheral hypopig-mentation; seen most frequently on thetrunk; repigmentation may take place overmonths or years, but lesion sometimesremains white indefinitely


Vitiligo; atypical mole; melanoma; tineaversicolor; lichen sclerosus; morphea; post-traumatic hypopigmentation


274 Halobetasol propionate

Therapy

None indicated for childhood lesions; surgi-cal excision for adult-onset lesions,although considered controversial

References

Zeff RA, Freitag A, Grin CM, Grant-Kels JM (1997) The immune response in halo nevi. Journal of the American Academy of Dermatology 37(4):620–624

Halobetasol propionate

�

Corticosteroids, topical, super potency

Halodermia

�

Knuckle pads

Halogenoderma

Synonym(s)

Bromoderma

;

iododerma

;

fluoroderma

Definition

Skin eruption resulting from exposure tobromide-containing drugs or substancessuch as potassium bromide (bromoderma),iodide-containing drugs or substances suchas water-soluble contrast media (iodo-derma), or fluoride-containing drugs orsubstances such as fluoride teeth gels(fluoroderma)

Pathogenesis

May represent a delayed hypersensitivityallergic response


Bromoderma: multiple, vegetative, ulcerat-ing and pustular plaques with elevated pap-illomatous borders, located mainly on thelegs, but also on the faceIododerma: vesicular, pustular, hemor-rhagic, suppurative, and/or ulcerativepapules and plaques occurring on the areasof skin with the highest concentration ofsebaceous glands, such as the faceFluoroderma: resembles iododerma, withnumerous and scattered papules and nod-ules


Tuberculosis; sarcoidosis; North Americanblastomycosis; rosacea; pyoderma gan-grenosum; acute febrile neutrophilic der-matosis; syphilitic gumma; pemphigus veg-etans

Therapy

Discontinuation of causative agent

�

References

Alagheband M, Engineer L (2000) Lithium and halogenoderma. Archives of Dermatology 136(1):126–127

Hanhart-Richner syndrome

�

Tyrosinemia II

Hansen disease

�

Leprosy

Hansen’s disease

�

Leprosy


Hartnup disorder 275

H

Harada syndrome

�

Vogt-Koyanagi-Harada syndrome

Harlequin baby

�

Ichthyosis fetalis

Harlequin fetus

�

Ichthyosis fetalis

Harlequin ichthyosis

�

Ichthyosis fetalis

Hartnup aminoaciduria

�

Hartnup disease

Hartnup disease

Synonym(s)

Hartnup disorder

,

Hartnup aminoaciduria

,

Hartnup syndrome

Definition

Disorder caused by defective transport ofneutral amino acids in the small intestineand kidney, resulting in a pellagra-like skineruption, cerebellar ataxia, and aminoaci-duria

Pathogenesis

Failure of the transport of tryptophan andother neutral alpha-amino acids in thesmall intestine and renal tubules; abnor-mality in tryptophan transport, leading toniacin deficiency that is responsible for pel-lagra-like eruption and photosensitivity


Gingivitis, stomatitis, glossitis; photosensi-tivity; multiple sun exposures leading todry, scaly, well-marginated plaques, resem-bling chronic eczema, affecting preferen-tially the forehead, cheeks, periorbitalregions, dorsal surface of the hands, andother light-exposed areas; vesiculobullouseruption with exudation sometimes occurs;hypopigmentation and/or hyperpigmenta-tion that is intensified with further sunlightexposure; intermittent cerebellar ataxiawith wide-based gait, spasticity, delayedmotor development, and tremulousness, allreversible with niacin therapy; diarrhea;attacks sometimes provoked by a febrile ill-ness, poor nutrition, sulfonamides, andpossibly emotional stress


Polymorphous light eruption; lupus ery-thematosus; atopic dermatitis; seborrheicdermatitis; nutritional pellagra; Cockaynesyndrome; carcinoid syndrome; ataxia tel-angiectasia; xeroderma pigmentosum

Therapy

Niacin 50–100 mg PO 3 times per day

�

;avoidance of sun exposure; high proteindiet

References

Kahn G (1986) Photosensitivity and photoderma-titis in childhood. Dermatologic Clinics 4(1):107–116

Hartnup disorder

�

Hartnup disease


276 Hartnup syndrome

Hartnup syndrome

�

Hartnup disease

Hashimoto-Pritzker disease

�

Congenital self-healing Langerhans cell histiocytosis

HAT

�

African trypanosomiasis

Haverhill fever

�

Rat-bite fever

Haxthausen’s disease

�

Cold panniculitis

Heat rash

�

Miliaria

Hebra’s disease

�

Erythema multiforme

Hecht-Beals syndrome

�

Beals-Hecht syndrome

Heloma

�

Clavus

Hemangiectasia hypertrophicans

�

Klippel-Trenaunay-Weber syndrome

Hemangioendothelioma

Synonym(s)

None

Definition

Varied group of proliferative and neoplas-tic vascular lesions, with a biological behav-ior falling somewhere between the benignhemangioma and malignant angiosarcoma

Pathogenesis

Unknown


Epithelioid hemangioendothelioma: solia-tary, sometimes painful, soft tissue mass,sometimes ulcerating, most commonly onthe lower extremitiesSpindle cell hemangioendothelioma: firmblue papules or nodules, often multifocalwithin given anatomic sites, occurring overthe distal extremitiesKaposiform hemangioendothelioma: usu-ally in retroperitoneum, but sometimes


Hemangiopericytoma 277

H

occurring in the skin; bluish papule or nod-ule; associated with consumption coagulop-athy and lymphangiomatosisRetiform hemangioendothelioma: slow-growing plaque with ill-defined borders,usually on the distal extremities

Differential diagnosisAngiosarcoma; Dabska tumor; Kaposi’s sar-coma; hemangioma


ReferencesGrezard P, Balme B, Ceruse P, Bailly C, Dujardin T,

Perrot H (1999) Ulcerated cutaneous epithelio-id hemangioendothelioma. European Journal of Dermatology 9(6):487–490

Hemangioma

Synonym(s)Angioma

DefinitionDense collections of dilated vessels occur-ring in the skin or internal organs

ReferencesDinehart SM, Kincannon J, Geronemus R (2001)

Hemangiomas: evaluation and treatment. Der-matologic Surgery 27(5):475–485

Hemangioma, capillary


Hemangioma, cavernous


Hemangioma, cherry

� Cherry hemangioma

Hemangiopericytoma

Synonym(s)None

DefinitionVascular sarcoma derived from pericytes,with distinctive histologic features and avariable course depending on the degree ofcellular atypia

PathogenesisUnknown

Clinical manifestationRapidly enlarging, asymptomatic, welldemarcated, soft or rubbery, red or bluishtumor; sessile or somewhat pedunculated;sometimes has surface lobularity or tel-angiectasis; located at one of many sites,including orbit, neck, mediastinum, epicar-dium, retroperitoneum, and upper andlower extremity; occurs in all age groups,but rare prior to the second decade or afterthe seventh decade

Differential diagnosisFibrous histiocytoma; malignant fibroushistiocytoma; synovial sarcoma; juxta-glomerular tumor; vascular leiomyoma;juvenile hemangioma; myxoid lipoma;myxoid liposarcoma; mesenchymal chond-rosarcoma

TherapyBland lesions with minimal mitotic activ-ity: wide local excision�; active and dys-plastic lesions: radical surgical excision,with or without adjunctive radiotherapy�


278 Hematoma

ReferencesPandey M, Kothari KC, Patel DD (1997) Haeman-

giopericytoma: current status, diagnosis and management. European Journal of Surgical Oncology 23(4):282–285

Hematoma

Synonym(s)None

DefinitionCollection of blood within soft tissue thatresults in swelling

ReferencesMcGillis ST, Ratner D, Clark R, Madani S, et al.

(1998) Atlas of excision and repair. Dermato-logic Clinics 16(1):181–194

Hemochromatosis

Synonym(s)Bronze diabetes, iron deposition disease,hereditary hemochromatosis; genetichemochromatosis; primary hemochroma-tosis

DefinitionAbnormal accumulation of iron in paren-chymal organs, leading to organ toxicity

PathogenesisAutosomal recessive trait; associated withtwo mutations in the HFE gene; error ofiron metabolism characterized by excessdietary iron absorption and iron deposi-tion in tissues; presence of free iron in bio-logical systems leads to rapid formation ofdamaging reactive oxygen metabolites,which can produce DNA cleavage, impairedprotein synthesis, and impairment of cellintegrity and cell proliferation, resulting incell injury and fibrosis

Clinical manifestationGeneralized hyperpigmentation; ichthyo-sis; skin atrophy; koilonychia; partial alo-pecia; diabetes mellitus; cirrhosis; conges-tive heart failure; hepatomegaly; splenome-galy; arthritis; amenorrhea; loss of libido;impotence; symptoms of hypothyroidism

Differential diagnosisAddison’s disease; polymorphous lighteruption; post-inflammatory hyperpigmen-tation; sun-induced tanning; drug-inducedhyperpigmentation; actinic reticuloid;poikiloderma of Civatte; argyria; iron over-load associated with chronic anemia; multi-ple blood transfusions; hyperplastic eryth-roid marrow from diseases such as heredi-tary sideroblastic anemias, severe alpha andbeta thalassemia; myelodysplastic syn-drome variants

TherapyPhlebotomy�; limiting of alcohol consump-tion; avoidance of iron supplements andraw oysters

ReferencesPowell LW (2002) Hereditary hemochromatosis

and iron overload diseases. Journal of Gastro-enterology & Hepatology 17 Suppl:S191–195

Hemorrhagic jaundice

� Leptospirosis

Henoch-Schönlein purpura

Synonym(s)Anaphylactoid purpura; Schönlein-Henochpurpura

DefinitionImmunoglobulin (Ig)A-mediated small-ves-sel vasculitis with involvement of the skin,


Hereditary angioedema 279

H

gastrointestinal tract, joints, and kidneys,occurring primarily in children

PathogenesisVascular deposition of IgA immune com-plexes, which activate complement compo-nents, which mediate tissue injury

Clinical manifestationProdrome of fever, anorexia, and headache;erythematous macules and papules on but-tocks and extremities, which become pur-puric; colic, vomiting, and diarrhea; polyar-thralgia; proteinuria and hematuria

Differential diagnosisUrticaria; lupus erythematosus; Churg-Strauss syndrome; essential mixed cry-oglobulinemia; polyarteritis nodosa; rheu-matoid arthritis; benign pigmented pur-pura; child abuse; bacterial endocarditis;meningococcemia; Rocky Mountain spot-ted fever

TherapyPrednisone; dapsone; azathioprine; intrave-nous immunoglobulin (IVIG)

ReferencesSaulsbury FT (2001) Henoch-Schonlein purpura.

Current Opinion in Rheumatology 13(1):35–40

Heparin necrosis

Synonym(s)None

DefinitionNecrotic areas of skin, usually at the site ofheparin injection, characterizing a local-ized hypersensitivity reaction

PathogenesisPossible immunologic basis

Clinical manifestationBegins as localized erythema, typically atheparin injection sites, usually in women;

burning pain; progression to bulla forma-tion and necrosis over a few days; morecommon in obese or diabetic patients

Differential diagnosisPyoderma gangrenosum; calciphylaxis; spi-der bite reaction; factitial disease; bacterialpyoderma; herpes simplex virus infection;fixed drug eruption

TherapyDiscontinuance of heparin therapy�;hydrocolloid dressings to ulcerated area;ulcer excision and skin grafting if ulcera-tion persists

ReferencesLevine LE, Bernstein JE, Soltani K, Medenica MM,

Yung CW (1983) Heparin-induced cutaneous necrosis unrelated to injection sites. Archives of Dermatology 119(5):400–403

Hepatic porphyria

� Porphyria cutanea tarda

Hepatolenticular degeneration

� Wilson disease

Hereditary angioedema

Synonym(s)None

DefinitionHereditary disorder characterized by pain-less, nonpruritic swelling of the skin

PathogenesisMutations in the C1-INH gene, transmittedas an autosomal dominant trait; two vari-


280 Hereditary baldness

ants: type I – low antigenic and functionalplasma levels of C1-INH protein; type II –presence of normal or elevated antigeniclevels of a dysfunctional mutant proteintogether with reduced levels of the func-tional protein; C1-INH deficiency permitsautoactivation of the first component ofcomplement (C1) with consumption of C4and C2

Clinical manifestationRecurrent, noninflammatory swelling of theskin and mucous membranes; erythema ormild urticarial eruption occasionally pre-ceding edema; sometimes precipitated bytrauma, anxiety, or stress; associated withlupus erythematosus and other autoim-mune diseases

Differential diagnosisChronic urticaria; pressure-induced urti-caria; acquired angioedema; ACE inhibitor-induced angioedema

TherapyAcute episodes: replacement with C1-INHconcentrates�; fresh-frozen plasma; proph-ylaxis: danazol 400–600 mg PO per day

ReferencesNzeako UC, Frigas E, Tremaine WJ (2001) Hered-

itary angioedema: a broad review for clini-cians. Archives of Internal Medicine 161(20):2417–2429

Hereditary baldness


Hereditary coproporphyria

Synonym(s)None

DefinitionOne of the porphyrias, characterized byabdominal pain, neuropsychiatric prob-lems, constipation, and skin changes

PathogenesisAutosomal dominant disease, resultingfrom defects in coproporphyrinogen oxi-dase; related to deposition of formed por-phyrins in the skin which become photoac-tive after sunlight exposure

Clinical manifestationSkin changes: blisters forming in sun-exposed areas; skin fragility; scarring;hypertrichosis in sun-exposed areasNeurologic changes: central nervous sys-tem signs, including seizures, mental statuschanges, cortical blindness, and coma;peripheral neuropathies predominantlymotor neuropathies; diffuse pain, espe-cially in the upper body; autonomic neu-ropathies, including hypertension and tach-ycardia; psychiatric abnormalities

Differential diagnosisPorphyria cutanea tarda; acute intermittentporphyria; adrenal crisis; biliary disease;fibromyalgia; Addison’s disease; acute abdo-men from diverse causes; psychosis; leadintoxication

TherapyGlucose 400 mg IV per day for mild attacks;hematin 4 mg per kg per day for 4 days foracute attacks�

ReferencesLim HW, Cohen JL (1999) The cutaneous porphy-

rias. Seminars in Cutaneous Medicine & Sur-gery 18(4):285–292

Hereditary hemochromatosis

� Hemochromatosis


Hermansky-Pudlak syndrome 281

H

Hereditary hemorrhagic telangiectasia

� Osler-Weber-Rendu syndrome

Hereditary hidrotic ectodermal dysplasia

� Hidrotic ectodermal dysplasia

Hereditary ichthyosis vulgaris


Hereditary leukokeratosis

� White sponge nevus

Hereditary osteo-onychodysplasia


Hereditary palmo-plantar keratoderma


Hereditary papulotranslucent acrokeratoderma


Hereditary symmetrical aplastic nevi of the temples

� Brauer’s syndrome

Heredofamilial angiomatosis


Heredopathia atactica polyneuritiformis

� Refsum disease

Herlitz syndrome


Hermansky-Pudlak syndrome

Synonym(s)None


282 Herpes gestationis

DefinitionOculocutaneous albinism associated with amild hemorrhagic diathesis

PathogenesisAutosomal recessive inheritance, many witha mutation of the HPS1 gene; storage poolplatelet defect with poor platelet aggrega-tion; accumulation of a ceroid lipofuscin inthe lysosomes of a variety of tissues

Clinical manifestationVariable degrees of hypopigmentation; pig-mented nevi and freckles common; mildbleeding disorder with epistaxis, easy bruis-ing, hemoptysis, gingival bleeding, andpostpartum bleeding; interstitial lung fibro-sis; restrictive lung disease; granulomatouscolitis

Differential diagnosisAlbinism; Chediak-Higashi syndrome

TherapyAvoidance of aspirin; low vision evaluationand rehabilitation; sun avoidance

ReferencesToro J, Turner M, Gahl, WA (1999) Dermatologic

manifestations of Hermansky-Pudlak syn-drome in patients with and without a 16-base pair duplication in the HPS1 gene. Archives of Dermatology 135(7)774–780

Herpes gestationis

Synonym(s)Pemphigoid gestationis; autoimmune der-matosis of pregnancy; pregnancy-associ-ated autoimmune disease

DefinitionAutoimmune bullous eruption developingin association with pregnancy

PathogenesisImmunoglobulin G (IgG) autoantibodiesproduced against bullous pemphigoid (BP)

antigen 2 (BPAG2) (also known as BP 180),which is component of the hemidesmo-some; trigger for autoantibody productionunknown

Clinical manifestationEruption develops during the second andthird trimesters; in 25% of patients, lesionsappear immediately after delivery, begin asintensely pruritic erythematous urticarialpatches and plaques, often periumbilical;lesions progress to tense vesicles and bul-lae, spreading peripherally, often sparingthe face, palms, soles, and mucous mem-branes; disease activity usually remitswithin days after parturition; some patientshave persistent disease activity that lastsmonths or years; sometimes recurs with theresumption of menses, use of oral contra-ceptives, and with subsequent pregnancies

Differential diagnosisBullous pemphigoid; linear IgA bullous der-matosis; dermatitis herpetiformis; herpessimplex virus infection; drug-induced bul-lous disorder; papular dermatitis of preg-nancy; prurigo gestationis of Besnier; pru-ritic urticarial papules and plaques of preg-nancy (PUPPP)

TherapyMild disease: corticosteroids, topical, highpotency.Severe disease: prednisone�

Herpes gestationis. Multiple vesicles and bullae on the upper extremities in a pregnant woman


Herpes simplex virus infection 283

H

ReferencesScott JE, Ahmed AR (1998) The blistering diseas-

es. Medical Clinics of North America 82(6):1239–1283

Herpes gladiatorum

� Herpes simplex virus infection

Herpes simplex virus infection

Synonym(s)None

DefinitionViral infection caused by Herpesvirus hom-inis (herpes simplex virus)

PathogenesisTransmitted through close personal con-tact; two viral subtypes: HSV-1 transmittedprimarily by contact with infected saliva;HSV-2 mainly transmitted sexually; afterdirect exposure to infectious material (i.e.,saliva, genital secretions), initial viral repli-cation occurs at either the skin or mucousmembrane entry site; after retrogradeaxonal flow from neurons at viral point ofentry and local replication, viral genomebecomes latent and no viral particles areproduced; stimulus (e.g., physical or emo-tional stress, fever, ultraviolet light) causesreactivation of the virus

Clinical manifestationNeonatal infection: onset of illness within24 hours of birth; most often, symptoms ofillness within the first week of life; rashnoted after symptoms begin; manifesta-tions of illness representative of the organsystems involved (i.e., CNS, lungs, gastroin-testinal tract, heart, kidneys); skin vesiclesdevelops on an erythematous base, whichmay coalesce into playues; localized eye

infection with conjunctival injection and awatery discharge; dendritic lesions on fluo-rescein staining of the cornea; acute gingi-vostomatitis: most frequent clinical presen-tation of first-episode, primary HSV infec-tion, although most patients haveasymptomatic first infection; fever (102–104°F); listlessness or irritability; inabilityto eat and/or drink; gingivitis with mark-edly swollen, erythematous, bleeding gums;occasional increased drooling noted ininfants; vesicular lesions develop on thetongue, buccal mucosa, and palate, withextension to lips and face; tender sub-mandibular or cervical adenopathy; dis-ease lasting from 3–7 days; recurrent orola-bial herpetic infection (herpes labialis):heralded by a prodrome of pain, tingling,burning, or itching, usually lasts up to6 hours; vesicular rash in crops of 3–5 vesi-cles, frequently arising near the vermillionborder; recurrences often associated withfebrile illnesses, local trauma, sun expo-sure, or menstruation; primary genitalinfections: most infections asymptomatic;severe constitutional symptoms: fever,malaise, myalgias, and occasional head-ache; vesicular rash; lesions sometimes per-sist for up to 3 weeks; painful inguinal lym-phadenopathy; dysuria; vaginal discharge;recurrent genital infections: vulvar irrita-tion and/or ulcerating or vesicular lesions;symptoms more severe in females; recur-rent infections in males sometimes presentwith vesicular lesions on the shaft of thepenis; local symptoms of recurrence: pain,itching, and dysuria; CNS infection:encephalitis possible manifestation of pri-mary or recurrent infection; other seque-lae: aseptic meningitis, transverse myelitis;herpetic whitlow (infection of a digit):presents with acute onset of edema, ery-thema, and localized pain and tendernessin the finger; associated fever and enlargedregional adenopathy; herpes gladiatorum:begins with painful vesicular lesions, fre-quently over the shoulders and neck inwrestlers (sites of skin-to-skin contact);Kaposi’s varicelliform eruption (eczemaherpeticum): clusters of umbilicated vesicu-lopustules in areas of a pre-existent derma-


284 Herpes zoster

titis; transmission occurs through contactwith an infected person or by dissemina-tion of primary or recurrent herpes; recur-rent episodes sometimes occur, but milderand not usually associated with systemicsymptoms; severe cases sometimes causescarring

Differential diagnosisImpetigo; candidiasis; varicella; herpeszoster; vesicular dermatophytosis; bullouspemphigoid; pemphigus vulgaris; aphthousstomatitis; Behçet’s disease; contact derma-titis

TherapyNeonatal infection, CNS infection: acyclo-vir�; first episode mucocutaneous infec-tion, recurrent mucocutaneous infection,herpetic whitlow, herpes gladiatorum: vala-cyclovir, famciclovir; chronic suppression:valacyclovir�; famciclovir

ReferencesSimmons A (2002) Clinical manifestations and

treatment considerations of herpes simplex vi-rus infection. Journal of Infectious Diseases 186 Suppl 1:S71–77

Herpes zoster

Synonym(s)Shingles; zoster

DefinitionNeurocutaneous infection caused by thevaricella-zoster virus, which occurs in peo-ple who have had chickenpox; represents areactivation of the dormant varicella-zostervirus

PathogenesisReactivation of dormant varicella-zostervirus (VZV); results most often from a fail-ure of the immune system to contain latentVZV replication; most commonly occurs inone or more posterior spinal ganglia or cra-nial sensory ganglia; trigger of reactivation

unclear, but some cases possibly related toexternal re-exposure to the virus, acute orchronic disease processes such as malig-nancies and other infections, medications,and emotional stress

Clinical manifestationMay begin with non-specific constitutionalsymptoms and signs; prodromal pain orparathesias along one or more der-matomes, lasting 1–10 days, followed bypatchy erythema in the dermatomal area ofinvolvement and regional lymphadenopa-thy; unilateral, grouped vesicles on ery-thematous base, with severe local pain; ves-icles initially clear, but eventually becom-ing pustular, rupturing, crusting, andinvoluting; scarring ensues if deeper epider-mal and dermal layers compromised byscratching, secondary infection, or othercomplicationsZoster oticus (geniculate zoster, zosterauris, Ramsay-Hunt syndrome, Hunt syn-drome): Ménière disease, Bell palsy, cer-brovascular accident or abscess of the ear;beginning with otalgia and herpetiformvesicles on the external ear canal, with orwithout features of facial paralysis, result-ing from facial nerve involvement, auditorysymptoms (e.g., deafness), and vestibularsymptomsDisseminated zoster: generalized eruptionof more than 15–25 extradermatomal vesi-cles, occurring 7–14 days after the onset ofdermatomal disease; occurs rarely in thegeneral population, but commonly in eld-erly, hospitalized, or immunocompromisedpatients; often an indication of depressedcell-mediated immunity caused by variousunderlying clinical situations, includingmalignancies, radiation therapy, cancerchemotherapy, organ transplants, andchronic use of systemic corticosteroids; dis-semination sometimes includes involve-ment of the lungs and central nervous sys-tem

Differential diagnosisVaricella; herpes simplex virus infection;impetigo; candidiasis; erysipelas; cellulitis;bullous pemphigoid; pemphigus; contact


Hidradenitis suppurativa 285

H

dermatitis; urticaria; photoallergic reac-tion; folliculitis; insect bite reaction; bra-chioradial pruritus

TherapyFamciclovir; valacyclovir; post-herpeticneuralgia prophylaxis: prednisone; post-herpetic neuralgia: capsaicin; tricyclic anti-depressants, such as amitriptyline: 25–100 mg PO daily; gabapentin: 300–2400 mgPO daily

ReferencesChen TM, George S, Woodruff CA, Hsu S (2002)

Clinical manifestations of varicella-zoster virus infection. Dermatologic Clinics 20(2):267–282

Herpetic whitlow

� Herpes simplex virus infection


Synonym(s)Suppurative hidradenitis; apocrine acne;apocrinitis

DefinitionDisorder of the terminal follicular epithe-lium in the apocrine gland–bearing skin,characterized by comedone-like follicularocclusion, chronic relapsing inflammation,mucopurulent discharge, and progressivescarring

PathogenesisUnknown disorder of follicular occlusion;earliest change: follicular plugging whichobstructs apocrine gland ducts; earliestinflammatory event: rupture of the follicu-lar epithelium: friction in intertriginouslocations considered possible contributingfactor; rupture followed by spillage of for-eign-body material into the dermis, initiat-ing an inflammatory response resulting in

foreign-body granuloma; bacterial infec-tion a risk factor for destructive scarring,but not a primary cause of the disease;genetic factors may be operative

Clinical manifestationHirsutism and obesity common findingsamong affected women; early symptoms ofpruritus, erythema, and local hyperhidro-sis; lesions occur in the axillae, groin area,nipples, and buttocks; painful and/or ten-der red papules and nodules; lesion healwith fibrosis and eventual recurrence in theadjacent area; painful or tender abscessesand inflamed, discharging papules or nod-ules; nodules coalesce and sometimesbecome infected, resulting in acuteabscesses; dermal contractures and rope-like elevation of the skin; multiple abscessesand sinus tracts form a subcutaneous hon-eycomb; double-ended comedones; associ-ated arthropathy sometimes presentingwith asymmetric pauciarticular arthritis,symmetric polyarthritis, or polyarthralgiasyndrome

Differential diagnosisGranuloma inguinale; lymphogranulomavenereum; actinomycosis; staphylococcalabscesses; Bartholin cyst; carbuncle; Crohndisease; infected or inflamed epidermoidcyst; tuberculosis; tularemia; ulcerative col-itis

TherapyWide surgical excision, preferably taking asmuch apocrine gland-bearing skin as possi-ble�; localized disease: surgical techniquesincluding incision and drainage; exteriori-zation; curettage; electrocoagulation of thesinus tracts; simple excision; triamcinolone3–5 mg per kg intralesionally to inflamednodules; tetracycline; erythromycin;isotretinoin; acitretin; dapsone

ReferencesBrown TJ, Rosen T, Orengo IF (1998) Hidradenitis

suppurativa. Southern Medical Association Journal 91(12):1107–1114


286 Hidradenoma, clear cell

Hidradenoma, clear cell


Hidradenoma papilliferum

Synonym(s)Papillary hidradenoma; hidradenoma vul-vae; apocrine adenoma; adenoma hidrade-noides

DefinitionBenign tumor with apocrine differentia-tion, most commonly seen in the genitalarea of women

PathogenesisUnknown

Clinical manifestationSolitary, well-circumscribed, firm-to-cystic,bluish papule or nodule, with occasionalulceration, usually noted in the vulvar areaof middle-aged women

Differential diagnosisLeiomyoma; epidermoid cyst; squamouscell carcinoma; hemangioma; pyogenicgranuloma; melanoma; Bartholin cyst


ReferencesVang R, Cohen PR (1999) Ectopic hidradenoma

papilliferum: a case report and review of the lit-erature. Journal of the American Academy of Dermatology 41(1):115–118

Hidradenoma vulvae


Hidroacanthoma simplex

� Poroma

Hidrocystoma, apocrine


Hidrocystoma, eccrine

� Eccrine hidrocystoma

Hidrotic ectodermal dysplasia

Synonym(s)Hereditary hidrotic ectodermal dysplasia;Clouston’s disease

DefinitionGenodermatosis characterized by nail dys-trophy, alopecia, and hyperkeratosis of thepalms and soles

PathogenesisAutosomal dominant trait; abnormal α-proteins in hair and nails

Clinical manifestationDystrophic nails; sparse, thin, fragile hair;thickening of the palms and soles; normalsweat function; skin dryness

Differential diagnosisAnhidrotic ectodermal dysplasia; pachony-chia congenita; Basan syndrome; chondr-oectodermal dysplasia; dyskeratosis con-genita


Histoplasmosis 287

H

TherapyNone

ReferencesChitty LS, Dennis N, Baraitser M (1996) Hidrotic

ectodermal dysplasia of hair, teeth, and nails: case reports and review. Journal of Medical Ge-netics 33(8):707–710

Hirsutism

DefinitionDevelopment of androgen-dependent ter-minal body hair in a woman at sites whereterminal hair not normally found

ReferencesMarshburn PB, Carr BR (1995) Hirsutism and vi-

rilization. A systematic approach to benign and potentially serious causes. Postgraduate Medi-cine 97(1):99–102, 105–106

His-Werner disease

� Trench fever

Histiocytic


Histiocytoid hemangioma


Histiocytoma

� Dermatofibroma

Histiocytoma cutis

� Dermatofibroma

Histiocytosis, Langerhans cell


Histiocytosis, regressing atypical


Histiocytosis X


Histoplasmosis

Synonym(s)Darling’s disease

Histoplasmosis. Crusted, infiltrated nodule on the leg


288 HIV-associated eosinophilic folliculitis

DefinitionPulmonary and systemic infection causedby the fungus Histoplasma capsulatum

PathogenesisAlveolar deposition caused by aerosoliza-tion of conidia and mycelial fragments fromcontaminated soil; susceptibility to dissemi-nation increased with impaired cellularhost defenses; intracellular conversion frommycelial to pathogenic yeast form aftermacrophage phagocytosis; clinical manifes-tations occur with continued exposure tolarge inocula; pulmonary infection may dis-seminate, with hematogenous spread

Clinical manifestationAcute pulmonary infection usually asymp-tomatic; with symptomatic disease, fever,headache, malaise, myalgia, abdominalpain, and chills; with exposure to largeinoculum, severe dyspnea may occur; non-specific signs of infection: erythema nodo-sum and erythema multiforme; occsionaljoint pain and infiltrated papules in theskinChronic pulmonary disease mostly inpatients with underlying pulmonary dis-ease; associated with cough, weight loss,fevers, and malaise; if cavitations present,hemoptysis, sputum production, andincreasing dyspnea.Progressive disseminated disease occursmostly in immunocompromised patients;skin lesions begins as small papules andulcerations; oropharyngeal ulcers some-times involve buccal mucosa, tongue, gin-giva, and larynx

Differential diagnosisBacterial or mycoplasma pneumonia; NorthAmerican blastomycosis; coccidioidomyco-sis; tuberculosis; sarcoidosis; aspergillosis;squamous cell carcinoma; lymphoma

TherapyNone for asymptomatic disease or for cuta-neous disease as sole sign of dissemination;progressive disease, particularly with men-ingitis – amphotericin B – 0.7–1 mg per kgper day IV to a total dose of 35 mg per kg�;

mildly symptomatic or prolonged acutepulmonary disease – ketoconazole; itraco-nazole

ReferencesMocherla S, Wheat LJ (2001) Treatment of histo-

plasmosis. Seminars in Respiratory Infections 16(2):141–148

HIV-associated eosinophilic folliculitis


HIV-related eosinophilic folliculitis


Hives

� Urticaria

Hoffman’s disease


Homocystinuria

Synonym(s)None

DefinitionInherited disorder of methionine metabo-lism


Hospital gangrene 289

H

PathogenesisThree main causes: (1) deficiency of cys-tathionine synthase; gene for this defi-ciency located on chromosomal band21q22.3; (2) insufficient vitamin B-12 syn-thesis resulting from a defect in the remeth-ylation of homocysteine to methionine;(3) deficiency in methylenetetrahydrofolatereductase; high concentrations of aminoacids that are competitive inhibitors oftyrosinase results in pigment dilution,regardless of the cause of increased homo-cystine levels

Clinical manifestationCutaneous findings: red macules on buccalmucosa; enlarged pores on the face; livedo-like pattern of blood vessels; atrophic scarson the arms and hands; multiple smallangiomas; hypopigmentation, which isreversible in patients with pyridoxine-responsive disease; coarse hair texture;hyperhidrosis; xerosis; acrocyanosis; Mar-fan’s-like habitus; generalized osteoporosis;arterial and venous thrombosis; mentalretardation; visual impairment

Differential diagnosisMarfan syndrome; thrombophlebitis

TherapyPyridoxine 300–600 mg PO per day;betaine 3 g PO twice daily in adults, 100 mgper kg PO per day in children less than3 years old as initial dose.Cyanocobalamin: 25–250 mcg PO per day;low methionine diet

ReferencesKabra M (2002) Dietary management of inborn

errors of metabolism. Indian Journal of Pediat-rics 69(5):421–426

Homogentisic acid oxidase deficiency

� Alcaptonuria� Ochronosis

Honeycomb atrophy


Hori nevus

� Nevus of Ota and Ito

Hori’s nevus


Hornet sting


Horse-collar neck


Horton disease


Hospital gangrene



290 Hot tub dermatitis

Hot tub dermatitis

� Hot tub folliculitis

Hot tub folliculitis

Synonym(s)Hot tub dermatitis; splash rash;pseudomonas folliculitis; whirlpool follicu-litis

DefinitionCommunity-acquired pseudomonas skininfection, resulting from bacterial coloniza-tion of hair follicles after exposure to con-tained, contaminated water

PathogenesisBacterial organism, Pseudomonas aerugi-nosa, found in soil and fresh water, gainsentry through hair follicles or via breaks inthe skin; predisposing factors: hot water,high pH (>7.8), and low chlorine level(<0.5 mg/L); predisposing environmentalconditions: prolonged water exposure,excessive numbers of bathers, inadequatepool care; risk factors: crowding, wearing ofsnug bathing suits, and frequency andduration of exposure

Clinical manifestationOnset usually about 48 hours after contami-nated water exposure; follicular papules,vesicles, and pustules, which may becrusted, on exposed skin, but usually spar-ing the face, neck, palms and soles; lesionsprogress to erythematous papules and pus-tules; clear spontaneously in 2–10 days;rarely recur; heal without scarring, butsometimes cause desquamation or hyper-pigmented macules; occasional mildaccompanying constitutional symptomsand signs

Differential diagnosisInsect bite reaction; inflammatory folliculi-tis; staphylococcal folliculitis; Grover’s dis-ease; pityriasis lichenoides et varioliformisacuta; scabies

TherapyNo effective therapy, including systemic ortopical antibiotics

ReferencesBhatia A, Brodell RT (1999) “Hot tub folliculitis”.

Test the waters – and the patient – for Pseu-domonas. Postgraduate Medicine 106(4):43–46

Howell-Evans syndrome

� Tylosis

Human African trypanosomiasis

� African trypanosomiasis

Human threadworm infection

� Strongyloidosis

Hunter syndrome

Synonym(s)Mucopolysaccharidosis type II

DefinitionInherited metabolic storage disease arisingfrom a deficiency of L-sulfoiduronate sulfa-tase


Hurler syndrome 291

H

PathogenesisX-linked trait; deficiency of L-sulfoiduro-nate sulfatase, which results in accumula-tion of mucopolysaccharides in the lyso-somes of the cells in the connective tissueand increase in their excretion in the urine

Clinical manifestationMild and severe form of disease; organsmost involved: bone, the various viscera,the connective tissue, and the brain; onsetby age 3 years; hirsutism; skin thickening,particularly over the fingers; multiple,ivory-white, pebbly papules or nodulesoverlying the scapula and near posterioraxillary fold; progressive deafness; hepat-osplenomegaly, joint stiffness; coarse facialfeatures; cardiovascular involvement

Differential diagnosisHurler syndrome; Sanfilippo syndrome;Scheie syndrome; Gaucher’s disease; osteo-genesis imperfecta; vitamin D-resistantrickets; Niemann-Pick disease

TherapyBone marrow transplantation; investiga-tional enzyme replacement therapy withiduronate-2-sulfatase

ReferencesPeters C, Krivit W (2000) Hematopoietic cell

transplantation for mucopolysaccharidosis IIB (Hunter syndrome). Bone Marrow Transplan-tation 25(10):1097–1099

Hurler syndrome

Synonym(s)Mucopolysaccharidosis type I-H

DefinitionInherited metabolic storage disease arisingfrom a deficiency of alpha-L-iduronidase

PathogenesisAutosomal recessive trait; deficiency ofalpha-L-iduronidase, which results in accu-mulation of mucopolysaccharides in thelysosomes of the cells in the connective tis-sue

Clinical manifestationOnset in early childhood; organs mostinvolved: the bone, the viscera, the connec-tive tissue, and the brain; lichenified, dry,thick skin with diminished elasticity;increased pigmentation on the dorsum ofthe hands; sclerodermalike changes; hyper-trichosis of the extremities; pale coloredhair; neurologic symptoms: hypertensivehydrocephalus syndrome, changes in thetonus of the musculature and the tendonreflex, and damage of the cranial nerves;myxedema in patients with associatedhypothyroidism; dwarfism; hepat-osplenomegaly; cardiovascular involve-ment; progressive deterioration of intellectafter a period of apparently normal devel-opment; speech disturbances; ocular symp-toms: progressive clouding of the cornea,megalocornea, hereditary glaucoma, andcongestion and atrophy of the optic disc;death often occurs before age 10 years fromprogressive neurologic and cardiovasculardeterioration

Differential diagnosisHunter syndrome; Sanfilippo syndrome;Scheie syndrome; Gaucher’s disease; osteo-genesis imperfecta; vitamin D-resistantrickets; Niemann-Pick disease

TherapyBone marrow transplantation; investiga-tional enzyme replacement therapy withalronidase

ReferencesWraith JE (2001) Enzyme replacement therapy in

mucopolysaccharidosis type I: progress and emerging difficulties. Journal of Inherited Met-abolic Disease 24(2):245–250


292 Hurler-Scheie syndrome

Hurler-Scheie syndrome

� Scheie syndrome

Hutchinson melanotic freckle

� Lentigo maligna

Hutchinson’s melanotic freckle

� Lentigo maligna

Hyalinosis cutis et mucosae

� Lipoid proteinosis

Hydroa herpetiformis


Hydrocortisone


Hydroquinone

Trade name(s)Solaquin Forte; Eldoquin; Eldopaque;Nuquin; Lustra; Melanex; Esoterica; Porce-lana Fade Cream; Esoterica; Tri-Luma


Drug classDepigmenting agent

Mechanism of actionInhibits enzymatic oxidation of tyrosine;suppresses other melanocytic metabolicprocesses

Dosage form1.5% cream (Eldopaque; Esoterica; Porce-lana); 2% cream (Nuquin); 3% lotion (Mela-nex); 4% cream (Solaquin Forte, Lustra);4% gel (Solaquin Forte); 4% cream withtretinoin and fluocinolone (Tri-Luma)


Hydroquinone. Dermatologic indications and dosage


Berloque dermatitis Apply 1–2 times daily Apply 1–2 times daily

Lentigo Apply 1–2 times daily Apply 1–2 times daily

Melasma Apply 1–2 times daily Apply 1–2 times daily


Apply 1–2 times daily Apply 1–2 times daily


Hydroxyurea 293

H

Common side effectsCutaneous: contact dermatitis, burningsensation, erythema

Serious side effectsCutaneous: ochronosis-like pigmentation



ReferencesGlaser DA, Rogers C (2001) Topical and systemic

therapies for the aging face. Facial Plastic Sur-gery Clinics of North America 9(2):189–196

Hydroxychloroquine

Trade name(s)Plaquenil


Drug classAntimalarial

Mechanism of actionNot completely elucidated; may work byimmunosuppressive effects, DNA binding,photo-protective effects, and/or anti-inflammatory mechanisms



Common side effectsCutaneous: exacerbation of psoriasis, blue-gray skin discoloration, transverse nailbands; skin eruptions

Gastrointestinal: nausea and vomiting,diarrheaLaboratory: elevated liver enzymesNeurologic: headache, nervousness, moodswings, vertigoOcular: halos, blurred vision

Serious side effectsHematologic: agranulocytosis, aplastic ane-miaNeurologic: seizuresOcular: visual changes from retinopathy


Contraindications/precautionsHypersensitivity to drug class or compo-nent; porphyria cutanea tarda; history ofretinal field changes

ReferencesVan Beek MJ, Piette WW (2001) Antimalarials.

Dermatologic Clinics 19(1): 147–160

Hydroxyurea

Trade name(s)Hydrea


Drug classCytotoxic agent

Mechanism of actionRibonucleotide reductase inhibition, whichis the rate-limiting enzyme in DNA synthe-sis

Dosage form500 mg tablets



294 Hydroxyurea

Hydroxychloroquine. Dermatologic indications and dosage



Start at 200 mg PO twice daily; titrate downward after a favorable response has occurred

Start at 3–5 mg per kg PO daily; titrate downward after a favorable response has occurred

Chronic graft versus host disease


12 mg per kg daily PO, divided into 2 doses

Dermatomyositis Start at 200 mg PO twice daily; titrate downward after a favorable response has occurred


Granuloma annulare Start at 200 mg PO twice daily; titrate downward after a favorable response has occurred





Lupus erythematosus, subacute cutaneous



Lymphocytic infiltration of the skin



Pemphigus foliaceus Start at 200 mg PO twice daily; titrate downward after a favorable response has occurred





Porphyria cutanea tarda

200 mg twice weekly for first month; gradually titrate dose upward over 3–6 months to 200 mg daily

3 mg per kg PO weekly for first month; gradually titrate dose upward over 3–6 months to 3 mg per kg PO daily

Reticular erythematous mucinosis



Sarcoidosis Start at 200 mg PO twice daily; titrate downward after a favorable response has occurred


Solar urticaria Start at 200 mg PO twice daily; titrate downward after a favorable response has occurred






Hymenoptera sting 295

H

Common side effectsCutaneous: stomatitis, alopecia, erythema,skin eruption, leg ulcersGastrointestinal: hepatitis, anorexia, nau-sea and vomiting, diarrhea, dyspepsiaNeurologic: headache, dizziness, hallucina-tions, seizuresRenal: renal insufficiency

Serious side effectsBone marrow: anemia, thrombocytopenia,leukopeniaNeoplastic: possible increased risk of leuke-miaPulmonary: pulmonary fibrosis

Drug interactionsBone marrow suppressants

Contraindications/precautionsHypersensitivity to drug class or compo-nent; bone marrow depression; pregnancy;caution in patients with impaired renalfunction or with other myelosuppressiveagents

ReferencesKumar B, Saraswat A, Kaur I (2001) Rediscovering

hydroxyurea: its role in recalcitrant psoriasis. International Journal of Dermatology 40(8):530–534

Hydroxyzine


Hymenoptera sting

Synonym(s)Bee sting, hornet sting, wasp sting, yellowjacket sting, fire ant bite

DefinitionReaction to a sting or bite from an insect oforder Hymenoptera, which includes bees,hornets, wasps, yellow jackets, and ants

PathogenesisOver 30 individual compounds contained invenom of winged Hymenoptera, includingbiogenic amines, polypeptides, protein tox-ins and enzymes; small amounts of lowmolecular weight protein contained invenom of fire or stinging ants; reactions toenvenomation either directly toxic (eitherlocal or systemic) or allergic (either local-ized or anaphylactic)

Clinical manifestationSimple sting: results in a local reaction withswelling and pain initially and itching a fewhours later; swelling sometimes increasesover several days and usually resolveswithin one weekImmediate hypersensitivity reaction: localswelling or urticaria with pain and itching;sometimes spreads to become more gener-alized, with urticaria, which may progressto involve the upper or lower airway in ananaphylactic reactionDelayed hypersensitivity reaction: eitherimmune-complex mediated (eitherimmunoglobulin M or immunoglobulin G)

Hydroxyurea. Dermatologic indications and dosage


Hypereosinophilic syndrome


Psoriasis 20–30 mg per kg PO daily 15–30 mg per kg PO daily

Reiter syndrome 20–30 mg per kg PO daily 15–30 mg per kg PO daily


296 Hyper-IgE syndrome

or systemic (serum sickness type) or local(Arthus type); occurs within 1 week ofenvenomation; symptoms: fever, arthral-gias and myalgias, headache, and generalmalaise; signs: eruption of either red mac-ules and papules or palpable purpura; jointswelling and tenderness with or withouteffusions; lymphadenopathy; glomerulitisor nephrotic syndromeFire ant bite: usually multiple; presents asswelling and pain with early vesicle forma-tion, followed by ulceration and possiblesecondary infection

Differential diagnosisAnaphylaxis from other causes; allergiccutaneous vasculitis; foreign body reac-tion; spider bite

TherapySimple envenomations: ice water soaks;pain control with ibuprofen or acetami-nophenGeneralized reactions: epinephrine: 0.15–0.3 mg SC or 0.2–1 mg IV, repeated every20–30 minutes if indicated�; diphenhy-dramine – 25-75 mg PO or IM or IV,repeated every 6 hours as needed�

ReferencesMetry DW, Hebert AA (2000) Insect and arachnid

stings, bites, infestations, and repellents. Pedi-atric Annals 29(1):39–48

Hyper-IgE syndrome

� Hyperimmunoglobulin E syndrome

Hypercare Certain-Dri


Hypercorticalism

� Cushing syndrome


Synonym(s)Idiopathic hypereosinophilic syndrome

DefinitionGroup of leukoproliferative disorders char-acterized by an overproduction of eosi-nophils, resulting in organ damage

PathogenesisUnderlying cause of eosinophil overpro-duction not well understood; cytokine-mediated eosinophils survive in the tissuesfor longer periods of time than normal,thus increasing the amount of tissue dam-age; cells contain granules that store toxiccationic proteins, the primary mediators oftissue injury; toxins: major basic protein,eosinophil peroxidase, eosinophil-derivedneurotoxin, and eosinophil cationic pro-tein; eosinophils also release specificcytokines that recruit additional eosi-nophils

Clinical manifestationCutaneous findings: pruritius; angioedema,urticaria, often with dermatographism; ery-thematous, pruritic papules, plaques andnodules, with or without ulcerationCardiac findings: chest pain, dyspnea, ororthopneaHematologic changes: splenomegaly;thrombotic episodesNeurologic findings: encephalopathy; cere-brovascular accidents or transient ischemicepisodes


Hyperimmunoglobulin E syndrome 297

H

Pulmonary changes: chronic, persistentcough, usually nonproductive; dyspneafrom congestive heart failure or pleuraleffusions; pulmonary fibrosisRheumatologic findings: arthralgias andmyalgias; occasional Raynaud phenomenonGastrointestinal findings: abdominal pain;nausea; diarrhea; hepatomegaly

Differential diagnosisAngiolymphoid hyperplasia with eosi-nophilia; atopic dermatitis; lupus ery-thematosus; drug reaction; parasitic infec-tion; malignancy with secondary eosi-nophilia; Churg-Strauss syndrome;eosinophilia-myalgia syndrome; eosi-nophilic fasciitis

TherapyPrednisone�; aggressive disease unrespon-sive to corticosteroids: hydroxyurea; vinc-ristine: 1–2 mg IV every 2 weeks; chloram-bucil: pulse of 4–10 mg/m2/d PO for 4 daysevery other month; interferon; photochem-otherapy for symptomatic control of skineruption and pruritus; bone marrow trans-plantation for life-threatening disease

ReferencesAssa'ad AH, Spicer RL, Nelson DP, Zimmermann

N, Rothenberg ME (2000) Hypereosinophilic syndromes. Chemical Immunology 76:208–229

Hyperhidrosis

DefinitionExcessive sweating of certain body areas,particularly axillae, palms, and soles

ReferencesTogel B, Greve B, Raulin C (2002) Current thera-

peutic strategies for hyperhidrosis: a review. European Journal of Dermatology 12(3):219–223


Synonym(s)Job syndrome; hyper-IgE syndrome, Job'ssyndrome

DefinitionHeritable disorder characterized by the pro-duction of high levels of the antibodyimmunoglobulin E (IgE), causing seriousskin and lung infections and atopic eczema-like eruption

PathogenesisAutosomal dominant trait; no clearlydefined defect of either T or B cell func-tion; chemotactic defect in neutrophils;activation of Th2 lymphocytes producingcytokines responsible for activation anddifferentiation of eosinophils

Clinical manifestationCharacteristic coarse facies; early non-spe-cific papular or pustular eruption, favoringthe scalp, proximal flexures, and buttocks;eczematous eruption; recurrent staphyloco-ccal abscesses, often described as coldabscesses because of lack of pain, heat, orredness; cellulitis; recurrent bronchitis,caused by S. aureus or Haemophilus influ-enzae; other systemic bacterial infections;chronic mucocutaneous candidiasis andonychomycosis; skeletal abnormalities: fre-quent painless bone fractures; scoliosis;hyperextensible joints

Differential diagnosisAtopic dermatitis; chronic mucocutaneouscandidiasis; recurrent folliculitis; staphylo-coccal carriage state with recurrent skininfections; DiGeorge syndrome; Wiskott-Aldrich syndrome; chronic granulomatousdisease; common variable immunodefi-ciency; X-linked hypogammaglobulinemia;leukocyte adhesion deficiency


298 Hyperkeratosis eccentrica

TherapyActive bacterial infection: nafcillin 500–2000 mg IV every 6 hours for 1–5 days,depending on therapeutic response; thendicloxacillin 500 mg PO 4 times daily for10–21 days, depending on therapeuticresponse; pediatric patient: 100–200 mg perkg IV per day in 4 divided doses for 1–5 days, depending on therapeutic response;then dicloxacillin 250 mg PO 4 times dailyfor 10–21 days, depending on therapeuticresponse; incision and drainage of fluctu-ant abscesses; fluconazole for active candi-diasis; cyclosporine; prophylaxis: cimeti-dine: 20–40 mg per kg PO 3–4 times perday; ascorbic acid: 500 mg PO per day; anti-bacterial soaps used 1–2 times per day

ReferencesShemer A, Weiss G, Confino Y, Trau H (2001) The

hyper-IgE syndrome. Two cases and review of the literature. International Journal of Derma-tology 40(10):622–628

Hyperkeratosis eccentrica

� Porokeratosis

Hyperkeratosis figurata centrifuga atrophicans

� Porokeratosis

Hyperkeratosis follicularis et parafollicularis in cutem penetrans

� Kyrle’s disease

Hyperkeratosis haemorrhagica

� Black heel


Synonym(s)Flegel disease; Flegel’s disease

DefinitionDisease of localized abnormal keratiniza-tion, characterized by inflammatory kera-totic papules

PathogenesisUltraviolet light possibly a factor

Clinical manifestationAsymptomatic, small, red-brown, hyperk-eratotic papules on the lower extremities,sparing the trunk; removal of the scalereveals bright red base, with pinpointbleeding

Differential diagnosisDisseminated superficial actinic porokera-tosis; porokeratosis of Mibelli; stucco kera-tosis; actinic keratosis; flat warts; acrokera-tosis verruciformis of Hopf; Kyrle’s disease

TherapyFluorouracil; acitretin; dermabrasion

ReferencesFathy S, Azadeh B (1988) Hyperkeratosis lenticu-

laris perstans. International Journal of Derma-tology 27(2):120–121


Hypohidrotic ectodermal dysplasia 299

H

Hyperkeratosis palmaris et plantaris


Hypermelanotic macule

� Café au lait macule

Hyperpituitarism

� Acromegaly

Hypertrichosis

DefinitionAbnormally increased growth of hair,regardless of location

ReferencesVashi RA, Mancini AJ, Paller AS (2001) Primary

generalized and localized hypertrichosis in children. Archives of Dermatology 137(7):877–884

Hypertrichosis lanuginosa acquisita

� Hypertrichosis lanuginosa

Hypertrichosis universalis congenita, Ambras type

� Ambras syndrome

Hypertrichotic osteochondrodysplasia

� Cantu syndrome

Hypertrophic morphea


Hypoadrenalism


Hypocorticism


Hypohidrotic ectodermal dysplasia

� Anhidrotic ectodermal dysplasia


300 Hypomelanosis guttata ideopathica

Hypomelanosis guttata ideopathica

� Idiopathic guttate hypomelanosis

Hypomelanosis of Cummins and Cottel


Hypomelanosis of Ito

Synonym(s)Incontinentia pigmenti achromians

DefinitionSyndrome characterized by hypopig-mented whorls of skin along the Blaschkolines

PathogenesisChromosomal mosaicism and sporadicmutations; identity of a specific gene notconfirmed

Clinical manifestationAsymmetric, hypopigmented or white mac-ules coalescing to form reticulated patchesalong the lines of Blaschko; macules cover-ing more than 2 dermatomes and often onboth sides of the body, present at birth;occasional associations with neurologic,skeletal, and other congenital abnormalities

Differential diagnosisIncontinentia pigmenti; nevoid hypermela-nosis; nevus depigmentosus; congenitalnevocellular nevus; post-inflammatoryhyperpigmentation

TherapyNone for pigmentary abnormality

ReferencesPinto FJ, Bolognia JL (1991) Disorders of hypopig-

mentation in children. Pediatric Clinics of North America 38(4):991–1017


I

Iatrogenic acrodermatitis enteropathica

�


IBIDS

�

Tay syndrome

Ichthyosiform erythroderma with vacuolation

�


Ichthyosiform nevus

�

CHILD syndrome

Ichthyosis

Synonym(s)

None

Definition

Groups of diseases represented by thick,scaly skin

References

Shwayder T (1999) Ichthyosis in a nutshell. Pedi-atrics in Review 20(1):5–12

Ichthyosis bullosa of Siemens

�


Ichthyosis congenita

�

Ichthyosis fetalis

Ichthyosis congenita larva

�

Lamellar ichthyosis

Ichthyosis fetalis

Synonym(s)


;

harlequin baby

;

ichthyosis congenita

;

keratosis diffusa feta-lis; harlequin fetus


302 Ichthyosis hystrix

Definition

Severe form of congenital ichthyosis, char-acterized by profound thickening of thekeratin layer in fetal skin, producing ahorny shell of platelike scale and contrac-tion abnormalities of the eyes, ears, mouth,and appendages

Pathogenesis

Probable autosomal recessive trait; abnor-mal lamellar granule structure and func-tion; abnormal conversion of profilaggrinto filaggrin


Condition present at birth; skin severelythickened with large, shiny plates of hyper-keratotic scale; deep fissures separate thescales; severe ectropion, leaving the con-junctiva at risk for desiccation and trauma;pinnae sometimes small and rudimentary,or absent; severe traction on lips causeseclabium and fixed open mouth; nasalhypoplasia and eroded nasal alae; limbsencased in the thick membrane, causingflexion contractures of the arms, legs, anddigits; limb motility poor or absent; hypo-plasia of the fingers, toes, and fingernails;temperature dysregulation; heat intoler-ance; occasional hyperthermia; restrictionof chest-wall expansion sometimes resultsin respiratory distress, hypoventilation, andrespiratory failure; dehydration from excesswater loss


Trichorrhexis invaginata; congenital ichthy-osiform erythroderma; lamellar ichthyosis;Conradi's disease; trichothiodystrophy;Sjogren-Larsson syndrome; X-linked ich-thyosis; lamellar ichthyosis; Netherton'ssyndrome

Therapy

Acitretin

References

Singh S, Bhura M, Maheshwari A, Kumar A, Singh CP, Pandey SS (2001) Successful treatment of harlequin ichthyosis with acitretin. Interna-tional Journal of Dermatology 40(7):472–473

Ichthyosis hystrix

�


Ichthyosis hystrix of Curth-Macklin

�


Ichthyosis, lamellar

�

Lamellar ichthyosis

Ichthyosis linearis circumflexa

�

Netherton syndrome

Ichthyosis nacrée

�

Ichthyosis vulgaris

Ichthyosis nigricans

�

X-linked ichthyosis

Ichthyosis nitida

�

Ichthyosis vulgaris


Id reaction 303

I

Ichthyosis palmaris et plantaris

�

Unna-Thost palmoplantar kerato-derma

Ichthyosis sebacea

�

Lamellar ichthyosis

Ichthyosis simplex

�

Ichthyosis vulgaris

Ichthyosis vulgaris

Synonym(s)

Common ichthyosis

;

autosomal dominantichthyosis

;

hereditary ichthyosis vulgaris

;

ichthyosis simplex; xeroderma

;

pityriasisvulgaris

;

ichthyosis nacrée

;

ichthyosisnitida

;

fish skin ichthyosis

Definition

Hereditary retention hyperkeratosis charac-terized by large, plate-like, scaly plaques

Pathogenesis

Autosomal dominant trait; altered profilag-grin expression leading to retained scale;chemical abnormality correlated withdecreased numbers of keratohyalin gran-ules


Symmetrical, variable scaling; small, fine,irregular, and polygonal scales, often curl-ing at the edges to give the skin a rough feel;

color ranging from white to dirty gray tobrown; most scaling occurring on extensorsurfaces of extremities, with sharp demar-cation between normal flexural folds andsurrounding affected areas; lower extremi-ties generally more affected than upperextremities; on trunk, scaling often morepronounced on back than abdomen; spar-ing of flexural folds; palmoplantar thicken-ing and hyperlinearity; relative sparing offace; improvement in summer or in warmclimate


X-linked ichthyosis; asteatosis; atopic der-matitis; lamellar ichthyosis; sarcoidosis;dermatophytosis; acquired ichthyosis

Therapy

Alpha hydroxy acids; emollients; keratolyt-ics such as salicylic acid; urea

References

Rabinowitz LG, Esterly NB (1994) Atopic dermati-tis and ichthyosis vulgaris. Pediatrics in Review 15(6):220–226

Ichthyosis, X-linked

�

X-linked ichthyosis

Ichthyotic neutral lipid storage disease

�


Id reaction

Synonym(s)

Autoeczematization

,

autosensitization


304 Idiopathic anetoderma of Schweninger and Buzzi

Definition

Acute, generalized reaction to a variety ofstimuli, including infections and inflamma-tory skin diseases

Pathogenesis

Unknown; theories of causation:(1) abnormal immune recognition of autol-ogous skin antigens; (2) increased stimula-tion of normal T cells by altered skin con-stituents; (3) dissemination of infectiousantigen with a secondary response; and(4) dissemination of cytokines from a pri-mary site


Acute onset of a pruritic, symmetrial, ery-thematous, papular or papulovesiculareruption, usually preceded by acute flare ofunderlying dermatitis or infection, at a sitedistant from the primary infection or der-matitis; vesicles sometimes present on thehands or feet; underlying conditions: der-matophytes, mycobacteria, viruses, bacte-ria, parasites, contact dermatitis, stasis der-matitis, or other eczematous processes


Atopic dermatitis; stasis dermatitis; sebor-rheic dermatitis; contact dermatitis; dys-hidrotic eczema; dermatophytosis; scabies;Gianotti-Crosti syndrome; pityriasislichenoides et varioliformis acuta; drugeruption; folliculitis

Therapy

Prednisone

�

; corticosteroids, topical,medium-potency

References

Gianni C, Betti R, Crosti C (1996) Psoriasiform id reaction in tinea corporis. Mycoses 39(7-8):307–308

Idiopathic anetoderma of Schweninger and Buzzi

�

Anetoderma

Idiopathic atrophoderma of Pasini and Pierini

�



Synonym(s)


;

hypomelanosis guttata ideopathica; leuko-dermia lenticular disseminata

;

leukopathiaguttata et reticularis symmetrica

;

seniledepigmented spots

;

symmetric progressiveleukopathy of extremities

Definition

Acquired, benign leukoderma, most com-monly seen in light-skinned women with ahistory of significant chronic sun exposure

Pathogenesis

Possibly related to sun exposure and itseffect on melanocytes; defect of the epider-mal melanin unit, resulting in hypopigmen-tation


Most commonly seen on the legs of fair-skinned, women, but also occurring on thedorsal aspect of the forearms; multiple,confetti-like, hypopigmented macules


Post-inflammatory hypopigmentation;scars; lichen sclerosus; vitiligo; tinea versi-color; flat warts; pinta

Therapy

Corticosteroids, topical, medium potency;tretinoin; cryosurgery; sun avoidance

References

Falabella R (1988) Idiopathic guttate hypomela-nosis. Dermatologic Clinics 6(2):241–247


Imiquimod 305

I

Idiopathic hypereosinophilic syndrome

�


Idiopathic hypertrophic osteoarthropathy

�


Idiopathic inflammatory myopathy

�

Dermatomyositis

Idiopathic lenticular mucocutaneous pigmentatio

�

Laugier-Hunziger syndrome

Idiopathic lobular panniculitis

�


Imiquimod

Trade name(s)

Aldara

Generic available

No

Drug class

Immunomodulator

Mechanism of action

Induction of cytokines, including tumornecrosis factor-

α

, interferon-

α

, interferon-

γ

, IL-1 and IL-6

Dosage form

5% cream


See table

Common side effects

Cutaneous:

burning sensation, irritant der-matitis, pruritus, local pain, hypopigmenta-tion

Imiquimod. Dermatologic indications and dosage


Basal cell carcinoma Apply 3 times weekly Not indicated


Apply every other day for 16 weeks Not indicated

Genital warts Apply 3 times weekly Not indicated

Keloid, post-excision Apply daily to excision site for 8 weeks

Not indicated


306 Immersion foot


None

Drug interactions

None



References

Dahl M (2002) Imiquimod: a cytokine inducer. Journal of the American Academy of Dermatol-ogy 47(9 suppl):205–208

Immersion foot

Synonym(s)

Trench foot

;

sea boot foot

;

paddy-field foot

;

tropical jungle foot

;

foxhole foot

Definition

Condition produced by prolonged expo-sure of the feet to non-freezing, moist,occlusive microenvironment

Pathogenesis

Hyperhydration causes maceration of thestratum corneum; aggravating factors: tightshoes, foot dependency, immobility, dehy-dration, trauma, history of peripheral vas-cular disease; cold exposure causesincreased blood viscosity, thrombosis,ischemia and cell injury

Clinical manifestationCold water immersion foot: pre-hyperemicstage with cyanotic, absent pulses, and cold,waxy feet; hyperemic stage with painfulfeet, bounding pulses, brawny edema; occurseveral hours after removing footwear;post-hyperemic stage with cold sensitivityand hyperhidrosis that lasts from weeks toyears; warm water immersion foot: severelypainful and/or pruritic, edematous, whitewrinkled feet, with sharp demarcationbetween involved and uninvolved skin

Differential diagnosisChilblains; Raynaud phenomenon; frost-bite; sweaty sock dermatitis; pitted keratol-ysis

TherapyBed rest, leg elevation, and drying of feet�

ReferencesWrenn K (1991) Immersion foot. A problem of the

homeless in the 1990s. Archives of Internal Medicine 151(4):785-788

Immune complex urticaria

� Urticarial vasculitis

Impetigo

Synonym(s)Impetigo contagiosa, Fox impetigo,impetigo bullosa, impetigo contagiosabullosa

DefinitionBacterial infection of the superficial layersof the epidermis caused by gram-positivebacterial pathogens

Impetigo. Exudative, eroded plaques with honey-colored crusts on the feet


Incontinentia pigmenti 307

I

PathogenesisBullous variant: gram-positive, coagulase-positive, group II Staphylococcus aureus,most often phage type 71; organisms oftenspread from nasal carriage siteNon-bullous variant: in the United States,group A beta hemolytic streptococcal infec-tion and Staphylococcus aureus occur withequal frequency; in other parts of theworld, group A beta hemolytic streptococ-cal infection is most common cause; organ-ism often transmitted through hand con-tact, entering through abraded or other-wise traumatized skin

Clinical manifestationBullous variant: acute onset of vesicles thatenlarge and quickly rupture, often leavinga peripheral collarette ofk scale; occurs inmilieu of hot and humid environmentswith crowded living conditions and poorhygieneNon-bullous variant: fragile vesicle or pus-tule that readily ruptures and becomes ahoney-yellow, adherent, crusted papule orplaque; located around the nose, mouth,and exposed parts of the body, sparing thepalms and soles; regional, tender lymphad-enopathy

Differential diagnosisHerpes simplex virus infection; varicella;dermatophytosis; pediculosis; thermal orchemical burn; erythema multiforme; fixeddrug reaction; arthropod bite reaction;incontinentia pigmenti; scabies; contactdermatitis; cutaneous candidiasis

TherapyBullous variant: dicloxacillin; cephalexin;mupirocin; bacitracinNon-bullous variant: dicloxacillin;cephalexin; erythromycin; mupirocin; baci-tracin



Impetigo bullosa

� Impetigo

Impetigo contagiosa

� Impetigo

Impetigo contagiosa bullosa

� Impetigo

Incontinentia pigmenti

Synonym(s)Bloch-Sulzberger syndrome, Bloch-Sie-mens syndrome

DefinitionHereditary disorder characterized by neu-rologic, ophthalmologic, dental, and cuta-neous abnormalities

Incontinentia pigmenti. Linear, hyperpigmented, verrucous plaques on the leg


308 Incontinentia pigmenti achromians

PathogenesisX-linked dominant, single gene disorder;mutations in NEMO/IKK-g, which encodesa critical component of the nuclear factor-B (NF-B) signaling pathway; patchy distri-bution of skin lesions resulting from tissuemosaicism due to random X-inactivation

Clinical manifestationCutaneous changes:Stage 1: linear, red papules and vesiclesgrouped on an erythematous base, mainlyon the extremitiesStage 2: linear, verrucous plaques on an ery-thematous baseStage 3: streaks and whorls of brown orslate-gray pigmentation along the lines ofBlaschko, particularly on the trunkStage 4: hypopigmented, atrophic, reticu-lated patches, mostly on the lower extremi-ties; lusterless, thin hair; nail dystrophy,ranging from mild pitting or ridging toseverely thickened, abnormally ridgednails; dental abnormalities• Ocular findings: retinal detachment; pro-liferative retinopathy; fibrovascular retro-lental membrane; cataracts; atrophy of theciliary body• Neurologic findings: seizures; develop-mental delay; mental retardation; ataxia,spasticity; microcephaly; cerebral atrophy;hypoplasia of the corpus callosum; periven-tricular cerebral edema

Differential diagnosisStage 1: bullous impetigo; herpes simplexvirus infection; varicella; epidermolysisbullosa; bullous mastocytosis; epidermo-lytic hyperkeratosis; erythema toxicumStage 2: linear epidermal nevus; lichenstriatus; X-linked dominant chondrodys-plasia punctataStage 3: linear and whorled nevoid hyper-melanosis; dermatopathia pigmentosareticularis; Naegeli-Franceschetti-Jadas-sohn syndromeStage 4: hypomelanosis of Ito; focal dermalhypoplasia syndrome

TherapyNone for skin abnormalities

ReferencesTomaraei SN, Bajwa RP, Dhiman P, Marwaha RK

(1995) Incontinentia pigmenti (Bloch-Sulz-berger syndrome): report of a case and review of the Indian literature. Indian Journal of Pedi-atrics 62(1):118–122

Incontinentia pigmenti achromians

� Hypomelanosis of Ito

Indian tick typhus


Infantile acropustulosis

� Acropustulosis of infancy

Infantile digital fibromatosis

Synonym(s)Digital fibrous tumor of childhood; Reyetumor; recurring digital fibroma of child-hood

DefinitionBenign, nodular proliferation of fibrous tis-sue occurring almost exclusively on thedorsal and lateral aspects of the fingers ortoes in infants and small children

PathogenesisUnknown

Clinical manifestationSingle or multiple, firm, erythematous,smooth, dome-shaped papules on the dor-sal-lateral aspect of distal phalanges of the


Infantile/childhood eosinophilic pustulosis of the scalp 309

I

fingers and toes; sparing of the thumbs andgreat toes; occasional spontaneous regres-sion

Differential diagnosisAcquired digital fibrokeratoma; wart;knuckle pad; dermatofibroma; granulomaannulare; angiofibroma; fibrosarcoma; xan-thoma; neurilemmoma; sarcoidosis

TherapySurgery only if impairment or deformity ofthe digits; triamcinolone 3–5 mg per ml int-ralesional

ReferencesKawaguchi M, Mitsuhashi Y, Hozumi Y, Kondo S

(1998) A case of infantile digital fibromatosis with spontaneous regression. Journal of Der-matology 25(8):523–526

Infantile eczema


Infantile eruptive papulous dermatitis


Infantile fibromatosis

� Juvenile fibromatosis

Infantile hemangioma


Infantile lichenoid acrodermatitis


Infantile myofibromatosis


Infantile papular acrodermatitis


Infantile scurvy


Infantile vegetating halogenosis


Infantile/childhood eosinophilic pustulosis of the scalp



310 Infection by achlorophillic algae

Infection by achlorophillic algae

� Protothecosis, cutaneous

Inflammatory angiomatous nodules


Inflammatory linear verrucous epidermal nevus

� Epidermal nevus

Insect bite reaction

� Papular urticaria

Interface parapsoriasis


Interferon-αααα

Trade name(s)Roferon A; Intron A

Generic availableNo

Drug classImmune modulator

Mechanism of actionAnti-viral; anti-proliferative; immunoregu-latory

Dosage formPowder for reconstitution for subcutaneousor intramuscular injection


Common side effectsGeneral: flu-like symptomsLaboratory: decreased white blood cellcount, elevated liver enzymes

Serious side effectsBone marrow: suppressionImmunologic: autoimmune thyroiditisNeurologic: spastic hemiplegia, mood dis-orders, seizures; peripheral neuropathyPulmonary: toxic effects

Drug interactionsBone marrow suppressants; vinca alka-loids; zidovudine; aminophylline; inter-leukin-2

Contraindications/precautionsHypersensitivity to drug class or compo-nent; pregnancy; autoimmune hepatitis

ReferencesEdwards L (2001) The interferons. Dermatologic

Clinics 19:139–146

Intertriginous inflammation

� Intertrigo

Intertrigo

Synonym(s)Intertriginous inflammation


Intravascular lymphomatosis 311

I

DefinitionSuperficial inflammation of skin caused bymoisture, bacteria, or fungi in the skin folds

ReferencesGuitart J, Woodley DT (1994) Intertrigo: a practi-

cal approach. Comprehensive Therapy 20(7):402–409

Intestinal amebiasis

� Amebiasis

Intra-oral fistula

� Oral cutaneous fistula

Intraepidermal adenocarcinoma


Intravascular endothelioma


Intravascular lymphomatosis


Interferon-αααα. Dermatologic indications and dosage


AIDS-associated Kaposi’s sarcoma

30 million IU/m2 subcutaneously or intramuscularly 3 times weekly

Not applicable

Basal cell carcinoma 500,000 IU subcutaneously 3 times weekly for 3 weeks

Not applicable

Behçet’s disease 2 million IU subcutaneously weekly, escalating to 12 million IU over 2 months

Not indicated

Cutaneous T cell lymphoma

1 million IU intralesional weekly for 4 weeks

Not indicated

Genital warts 250,000 IU intralesional twice weekly for 8 weeks

Not indicated


250,000 IU intralesional twice weekly for 8 weeks

Not applicable

High risk melanoma adjuvant therapy

20 million IU per m2 IV 5 days weekly for 4 weeks, followed by 10 million IU per m2 subcutaneously 3 times weekly for 48 weeks


Infantile hemangioma Not applicable 3 million IU subcutaneously daily for up to 18 months

Keloid, post-excision 1.5 million IU intralesional twice over 4 days

1.5 million IU intralesional twice over 4 days

Squamous cell carcinoma

500,000 IU subcutaneously 3 times weekly for 3 weeks

Not applicable


312 Inverted follicular keratosis

Inverted follicular keratosis

Synonym(s)None

DefinitionBenign proliferation characterized by endo-phytic growth and histologic follicular dif-ferentiation

PathogenesisUnknown

Clinical manifestationSolitary, skin-colored papule or nodulewith a smooth or minimally keratotic sur-face, most commonly on the face of middle-aged patients

Differential diagnosisSeborrheic keratosis; wart; squamous cellcarcinoma; keratoacanthoma; basal cell car-cinoma


ReferencesSoylu L, Akcali C, Aydogan LB, Ozsahinoglu C,

Tuncer I (1993) Inverted follicular keratosis. American Journal of Otolaryngology 14(4):247–248

Iododerma

� Halogenoderma

Iron deposition disease

� Hemochromatosis

Ischemic ulcer

� Decubitus ulcer

Isotretinoin

Trade name(s)Accutane; Amnesteem; Sotret

Generic availableNo

Drug classRetinoid

Mechanism of actionInhibition of sebaceous gland differentia-tion and proliferation; reduction in seba-ceous gland activity; normalization of fol-licular epithelial differentiation

Dosage form10 mg, 20 mg, 40 mg capsule


Common side effectsDermatologic: peeling on hands and feet,cheilitis, skin fragility, alopecia, dry skin,pruritus, paronychiaEyes: dry eyes, with contact lens intoler-ance; dry mucous membranesMusculoskeletal: myalgias, arthralgiasLaboratory: hyperlipidemia

Serious side effectsEye: decreased night visionNeurologic: spinal hyperostosis, pseudotu-mor cerebri, mood disorderGastrointestinal: hepatotoxicity, pancreati-tis


Isotretinoin 313

I

Isotretinoin. Dermatologic indications and dosage


Acne conglobata 0.5 mg/kg PO daily for 1 month followed by 1 mg/kg daily for 4 months

0.5 mg/kg PO daily for 1 month followed by 1 mg/kg daily for 4 months

Acne necrotica 0.5 mg/kg PO daily for 1 month followed by 1 mg/kg daily for 4 months


Acne vulgaris 0.5 mg/kg PO daily for 1 month followed by 1 mg/kg daily for 4 months



0.5 mg/kg PO daily for 1 month followed by 1 mg/kg daily for 4–6 months


Chloracne 0.5 mg/kg PO daily for 1 month followed by 1 mg/kg daily for 4 months


Darier disease 0.2–0.3 mg/kg PO daily for 1 month, followed by 0.5–1.0 mg/kg daily indefinitely

0.2–0.3 mg/kg PO daily for 1 month, followed by 0.5–1.0 mg/kg daily indefinitely

Dissecting cellulitis of the scalp






Gram negative folliculitis






Keratosis pilaris atrophicans



Lamellar ichthyosis 0.2–0.3 mg/kg PO daily for 1 month, followed by 0.5–1.0 mg/kg daily indefinitely


Lichen sclerosus 0.5 mg/kg PO daily for 1 month followed by 1 mg/kg daily for 4 months





Muir-Torre syndrome 0.5–1.0 mg per kg PO indefinitely 0.5–1.0 mg per kg PO indefinitely


314 Itching purpura of Loewenthal

Genitourinary: major birth defects; pseu-dotumor cerebri

Drug interactionsTretinoin; benzoyl peroxide; car-bamazepine; tetracyclines

Contraindications/precautionsHypersensitivity to drug class or compo-nent; pregnancy; caution in patients withrenal or hepatic dysfunction, history ofpancreatitis or diabetes mellitus; childrenmay be more sensitive to effects on bones,which may prevent normal bone growthduring puberty

ReferencesHirsch RJ, Shalita AR (2001) Isotretinoin dosing:

past, present, and future trends. Seminars in Cutaneous Medicine & Surgery 20(3):162–165

Itching purpura of Loewenthal


Ito, nevus of


Itraconazole

Trade name(s)Sporanox

Generic availableNo

Drug classAzole antifungal agent


Dosage form100 mg tablet; 10 mg per ml oral solution

Pityriasis rubra pilaris 0.5 mg/kg PO daily for 1 month followed by 1 mg/kg daily for 4 months





Rosacea 10–20 mg PO daily for 4–6 months Not indicated

Sebaceous gland hyperplasia

10–20 mg PO daily or every other day indefinitely

Not indicated

Steatocystoma mutiplex



T-cell lymphoma, cutaneous

1 mg per kg PO daily for 4–6 months 1 mg per kg PO daily for 4–6 months

Transient acantholytic dermatosis

0.5–1.0 mg per kg PO daily for 4–5 months

Not applicable

Isotretinoin. Dermatologic indications and dosage (Continued)



Itraconazole 315

I


Common side effectsCutaneous: skin eruption, vasculitisGastrointestinal: nausea and vomiting,diarrhea, dyspepsia

Laboratory: elevated liver enzymes, hyper-triglyceridemia

Serious side effectsCutaneous: anaphylaxis, Stevens-Johnsonsyndrome reactionGastrointestinal: hepatotoxicity

Itraconazole. Dermatologic indications and dosage


Aspergillosis 200 mg PO daily until clearing Not indicated

Chromoblastomycosis 200 mg twice daily one week per month for 7 months

Not established

Eumycetoma 300 mg PO daily for months to years Not established

Histoplasmosis 200–400 mg PO daily for 6–12 months

3–5 mg per kg PO once daily for 6–12 months

Majocchi granuloma 200 mg PO daily for 4–6 weeks 5 mg per kg PO once daily for 4–6 weeks

North American blastomycosis

200–400 mg PO daily for a minimum of 6 months

5–7 mg per kg PO daily for a minimum of 6 months

Onychomycosis 200 mg PO twice daily one week per month for 3 months

5 mg per kg once daily for 7 consecutive days each month for 3 months

Oropharyngeal candidiasis

200 mg PO daily for 1–2 weeks 5 mg per kg PO once daily for 1–2 weeks

Protothecosis 200 mg PO daily for 2–6 weeks Not established

South American blastomycosis

100 mg PO daily for 6 months 5–7 mg per kg PO daily or divided into 2 doses for 6 months

Sporotrichosis, disseminated

200 mg PO twice daily indefinitely 5 mg per kg PO daily indefinitely

Sporotrichosis, lymphocutaneous variant

100 mg PO twice daily for 4–8 weeks; if no obvious improvement or if evidence of progressive fungal disease occurs, increase dose in 100 mg increments

100 mg PO daily; contintue for at least 1 week following clinical resolution

Tinea capitis 200 mg PO daily for 1–3 weeks 5 mg per kg PO daily for 2–4 weeks

Tinea corporis 200 mg PO daily for 1–3 weeks 5 mg per kg PO once daily for 1–3 weeks

Tinea cruris 200 mg PO daily for 1–3 weeks 5 mg per kg PO once daily for 1–3 weeks

Tinea faciei 200 mg PO daily for 1–3 weeks 5 mg per kg PO once daily for 1–3 weeks

Tinea pedis 200 mg PO daily for 1–3 weeks 5 mg per kg PO once daily for 1–3 weeks

White piedra 100 mg daily until culture-negative Not established


316 Ivermectin

Drug interactionsAmiodarone; amitriptyline; antacids; bar-biturates; buspirone; carbamazepine;cyclosporine; digoxin; glyburide/met-formin; protease inhibitors; phenytoin;pimozide; quinidine; rifampin; statins; sul-fonylureas; tacrolimus; theophylline; vincaalkaloids; warfarin

Contraindications/precautionsHypersensitivity to drug class or compo-nent; use of the following medications – cis-apride, midazolam, triazolam, pimozide,quinidine, dofetilide, lovastatin, simvasta-tin; history of congestive heart failure; cau-tion in patients with cardiovascular or pul-monary disease or impaired liver or renalfunction

ReferencesMoosavi M, Bagheri B, Scher R (2001) Systemic

antifungal therapy. Dermatologic Clinics 19(1):35–52

Ivermectin

Trade name(s)Stromectol



Mechanism of actionIncreases nerve and muscle cell permeabil-ity of targetpathogens



Common side effectsCutaneous: pruritus, skin eruption, edemaLymph nodes: lymphadenopathyNeurologic: dizziness




Referencesdel Giudice P (2002) Ivermectin in scabies. Cur-

rent Opinion in Infectious Diseases 15(2):123–126

Ivermectin. Dermatologic indications and dosage



200 mcg per kg PO for 1 dose, repeat in 10 days


Onchocerciasis 150 mcg per kg PO for 1 dose 150 mcg per kg PO for 1 dose

Scabies 200 mcg per kg PO for 1 dose, repeat in 10 days


Strongyloidosis 200 mcg per kg PO for 1 dose, repeat in 10 days



J

Jacob’s ulcer

�

Basal cell carcinoma

Jadassohn-Lewandowsky syndrome

�

Pachyonychia congenita

Jessner’s lymphocytic infiltrate

�

Jessner lymphocytic infiltration of skin

Jessner’s lymphocytic infiltration of skin

Synonym(s)

Jessner’s lymphocytic infiltrate

,

lymphocytic infiltrate of Jessner

;

benignchronic T-cell infiltrative disorder

Definition

Chronic benign T-cell infiltrative process ofthe skin

Pathogenesis

Possibly a photosensitivity disorder


One or a few asymptomatic, erythematouspapules, which expand peripherally to formwell demarcated, infiltrated, red plaques,usually on sun-exposed skin; occasional-spontaneous resolution after severalmonths


Lupus erythematosus; polymorphous lighteruption; granuloma faciale; lymphoma;cutaneous metastasis; granuloma annulare;sarcoidosis; fixed drug eruption

Therapy

Triamcinolone 3–4 mg per ml intralesional;corticosteroids, topical, high potency;hydroxychloroquine; prednisone; thalido-mide; surgical excision of individuallesions; superficial orthovoltage radiation;cryotherapy

References

Guillaume JC, Moulin G, Dieng MT, Poli F, Morel P, et al. (1995) Crossover study of thalidomide vs placebo in Jessner’s lymphocytic infiltration of the skin. Archives of Dermatology 131(9):1032–1035

Jeunes filles, des

�

Acne excoriée


318 Job syndrome

Job syndrome

�


Job's syndrome

�


Jock itch

�

Tinea cruris

Jogger’s nipples

Synonym(s)

None

Definition

Irritation of the nipples secondary to fric-tional trauma from clothing worn by run-ners

Pathogenesis

Frictional trauma on sensitive skin fromhard shirt fabrics


Soreness, dryness, erythema, erosions, andbleeding of the nipples, worse in those witherect nipples; occurs in women who do notwear bras when running or in men whowear shirts made of hard, synthetic fibers


Contact dermatitis; atopic dermatitis;xerotic eczema; Paget’s disease of the nipple

Therapy

Protective bras in women

�

; soft-fiber outergarments, made of materials such as silk;

protective tape over nipples before run-ning; emollient creams

References

Ramsey ML (1997) Skin care for active people. Physician and Sportsmedicine 25(3):131–132

Junctional epidermolysis simplex

�


Juvenile fibromatosis

Synonym(s)


Definition

Group of disorders of infancy and child-hood, characterized by proliferation offibroblasts

Pathogenesis

Unknown; juvenile hyaline fibromatosisvariant a disorder of glycosaminoglycansynthesis


Infantile myofibromatosis: one or multiple,rubbery or hard, skin-colored papules,either superficial or deep to the muscle,most commonly occurring on the head,neck, and trunk; usually present at birth orwithin the first few months of life; regres-sion by age 2 years; viscera rarely involved,but if so, prognosis is poorFibrous hamartoma of infancy: usuallypresent at birth, often in the axillary area,shoulder or groin region; presents asenlarging subcutaneous nodule; occasionalspontaneous resolutionJuvenile hyaline fibromatosis (systemichyalinosis): onset in early infancy with mul-tiple, hard or soft, fixed or mobile, translu-cent papules and nodules of the scalp, face,


Juvenile xanthoma 319

J

gingivae, neck and trunk; osteolytic lesionsof skull, long bones, or phalanges; poormuscle development; joint contractures inadult lifeInfantile digital fibromatosis: multiple,firm, smooth, pink or flesh-colored papulesof the fingers or toes, at birth or early child-hood, often with spontaneous regressionafter 2–3 years


Acquired digital fibrokeratoma; granulomaannulare; angiofibroma; fibrosarcoma; leio-myoma; leiomyosarcoma; juvenile xan-thogranuloma; sarcoidosis; multicentricreticulohistiocytosis; knuckle pads

Therapy

Infantile myofibromatosis: none indicated iflimited to superficial structures; chemo-therapy if visceral involvementFibrous hamartoma of infancy: surgicalexcisionJuvenile hyaline fibromatosis: no effectivetherapyInfantile digital fibromatosis: excisionalsurgery only if impairment or deformity ofthe digits

References

Campbell RJ, Garrity JA (1991) Juvenile fibroma-tosis of the orbit: a case report with review of the literature. British Journal of Ophthalmolo-gy 75(5):313–316

Juvenile giant cell granuloma

�

Juvenile xanthogranuloma

Juvenile hyaline fibromatosis

�

Juvenile fibromatosis


Synonym(s)

Nevoxanthoendothelioma

;

xanthoma mul-tiplex

;

juvenile xanthoma

;

congenital xan-thoma tuberosum

;

xanthoma naviforme

;

juvenile giant cell granuloma

Definition

Benign papules and nodules, composed ofhistiocytic cells, that predominantly occurin infancy and childhood

Pathogenesis

Possibly a granulomatous reaction of histi-ocytes to an unknown stimulus


Occurs in infancy or early childhood, withasymptomatic, smooth, firm papules thatinitially are red-brown, then quickly changecolor to yellow, usually on the trunk orupper extremities; lesions resolve spontane-ously in months to years, leaving small,atrophic scars


Xanthoma; mastocytoma; insect bite reac-tion; granuloma annulare; sarcoidosis; Spitznevus; Langerhans cell histiocytosis; non-Langerhans cell histiocytosis; benigncephalic histiocytosis; generalized eruptivehistiocytoma; self-healing reticulohistiocy-toma; xanthoma disseminatum

Therapy

Excision for cosmetic reasons only

References

Chang MW (1999) Update on juvenile xan-thogranuloma: unusual cutaneous and system-ic variants. Seminars in Cutaneous Medicine & Surgery 18(3):195–205

Juvenile xanthoma

�



320 Juxtaepidermal poroma

Juxtaepidermal poroma

�

Poroma


K

K-M syndrome

�


Kaltostat

�

Alginates

Kaposi sarcoma

�

Kaposi’s sarcoma

Kaposi varicelliform eruption

�

Eczema herpeticum

Kaposi’s dermatosis

�


Kaposi’s sarcoma

Synonym(s)

Kaposi sarcoma

;

multiple idiopathic hem-orrhagic sarcoma

Definition

Neoplasm of endothelial origin, involvingthe skin, mucosal surfaces, and internalorgans

Pathogenesis

Unclear whether a hyperplastic disease or atrue neoplasm; Herpes hominis virus-8(HHV-8) linked to all subtypes; co-factors:immunosuppression, genetics, country ofresidence, and male sex


Classic subtype: usually affects older menof Mediterranean or eastern European

Kaposi’s sarcoma.

Violaceous papules and plaques on the lower extremity


322 Kaposi’s sarcoma-like granuloma

backgrounds; sometimes arises in chroni-cally edematous extremities; violaceouspatches, plaques, or nodules on the lowerextremities, which can be painful and canulcerateAfrican endemic subtype: primarily affectsboys and men; appears same as classic sub-type or in a more deadly form involvingbones and lymph systemIatrogenic subtype: seen in kidney and livertransplant patients on immunosuppressivedrugs; usually regresses after immunosup-pressive drug stoppedAIDS-related subtype: lesions often appearon the upper body, including the oral cav-ity, head, neck, back, and in viscera; beginas discrete, red or purple patches that arebilaterally symmetric and initially tend toinvolve the lower extremities; patchesbecome elevated, evolving into nodules andplaques; sometimes arise as a large infiltrat-ing mass or as multiple, cone-shaped, fria-ble tumors


Pyogenic granuloma; tufted angioma;melanocytic nevus; melanoma; cavernoushemangioma; angiokeratoma; metastasis;myofibromatoma; arteriovenous malforma-tions

Therapy

None indicated for indolent skin tumors inelderly patients; localized disease: cryother-apy; radiation therapy; surgical excision orlaser ablation; intralesional vinblastinechemotherapy; disseminated disease: vin-blastine 3.5–10 mg IV weekly, or chemo-therapy combinations, with vinblastine,bleomycin, and doxorubicin; AIDS-associ-ated disease: antiviral therapy

References

Geraminejad P, Memar O, Aronson I, Rady PL, Hengge U, Tyring SK (2002) Kaposi's sarcoma and other manifestations of human herpesvi-rus 8. Journal of the American Academy of Der-matology 47(5):641–655

Kaposi’s sarcoma-like granuloma

�


Kaposi’s varicelliform eruption

�

Herpes simplex virus infection


Synonym(s)

K-M syndrome

;

consumptive thrombocy-topenia

;

giant hemangioma syndrome

Definition

Thrombocytopenia caused by sequestra-tion and destruction of platelets in a largevascular lesion, usually a cavernous heman-gioma

Pathogenesis

Vascular lesion cause platelet trapping andactivation, with consumption of coagula-tion factors


Presents as a reddish-brown skin plaque ornodule that progresses to a large violaceousmass; petechiae, bruising, and bleeding;high-output cardiac failure; may occur incavernous hemangioma, Kaposi heman-gioendothelioma, or tufted angioma


Coagulation abnormality of other cause;angiosarcoma; port-wine stain; congenitalhemangiopericytoma; kaposiform heman-


Keloid 323

K

gioendothelioma of infancy and childhood;teratoma; lymphatic malformation; venousmalformation; infantile fibrosarcoma;infantile myofibromatosis; congenitalhemangiopericytoma; epithelioid heman-gioendothelioma

Therapy

Prednisone; interferon; hematologic agentssuch as epsilon aminocaproic acid, aspirin,and dipyridamole, pentoxifylline, and cryo-precipitate

References

Hall GW (2001) Kasabach-Merritt syndrome: pathogenesis and management. British Journal of Haematology 112(4):851–862

Kawasaki disease

Synonym(s)

Mucocutaneous lymph node syndrome

;

Kawasaki syndrome; acute febrile mucocu-taneous lymph node syndrome

Definition

Acute systemic vasculitis associated with afebrile illness; skin and mucous membraneinvolvement

Pathogenesis

May be caused by a ubiquitous infectiousagent in certain genetically predisposedindividuals


Prolonged fever; polymorphous exanthem;swelling and induration of the hands andfeet, with subsequent desquamation; non-exudative conjunctival injection; hemor-rhagic, dry, fissured lips; “strawberrytongue”; non-suppurative cervical lym-phadenopathy; myocarditis and pancardi-tis; coronary artery abnormalities; arthral-gias and arthritis; urethritis with sterilepyuria; aseptic meningitis; diarrhea, vomit-ing, abdominal pain; hydrops of the gall-

bladder; auditory abnormalities; testicularswelling, pneumonitis


Viral exanthem; erythema multiforme;scarlet fever; rubeola; staphylococcalscalded skin syndrome; Stevens-Johnsonsyndrome/toxic epidermal necrolysis; lept-ospirosis; Rocky Mountain spotted fever;acrodynia; juvenile rheumatoid arthritis;polyarteritis nodosa

Therapy

Intravenous immunoglobulin (IVIG), 2 gper kg, as a single infusion over 10–12hours

�

; aspirin 80–100 mg per kg per dayPO in 4 divided doses until the fever hasabated for several days

References

Rowley AH, Shulman ST (1999) Kawasaki syn-drome. Pediatric Clinics of North America 46(2):313–329

Kawasaki syndrome

�

Kawasaki disease

Kelley-Seegmiller syndrome

�

Lesch-Nyhan syndrome

Keloid

Synonym(s)

Cheloid

Definition

Overgrowth of fibrous tissue that usuallydevelops at the site of a skin injury, where


324 Keratinous cyst

the tissue extends beyond borders of theoriginal wound, usually does not regressspontaneously, and tends to recur afterexcision

Pathogenesis

Probable genetic factors; imbalancebetween the anabolic and catabolic phasesof healing process; more collagen producedthan degraded


Rubbery or hard, reddish-brown papule ornodule, with regular margins; some withclawlike pseudopods extending beyond theareas of trauma, projecting above the levelof the surrounding skin; no spontaneousregression; lesion become less red overmany months or years; most common loca-tions: earlobes, face, neck, lower extremi-ties, breast, chest, back, and abdomen


Hypertrophic scar; squamous cell carci-noma; dermatofibroma; dermatofibrosar-coma protuberans; fibromatosis; NorthAmerican blastomycosis

Therapy

Triamcinolone 10–20 mg per ml intrale-sional; cryotherapy; silicone gel sheet; com-pression dressing; superficial orthovoltageradiation therapy; surgical excision withpostoperative interferon or imiquimod

References

Shaffer JJ, Taylor SC, Cook-Bolden F (2002) Keloi-dal scars: a review with a critical look at thera-peutic options. Journal of the American Academy of Dermatology46(2):S63–97

Keratinous cyst

�

Pilar cyst

Keratoacanthoma

Synonym(s)

Self-healing squamous cell carcinoma

;

self-healing epithelioma

Definition

Low-grade malignancy of the piloseba-ceous epithelium, characterized by rapidgrowth over a few weeks to months, fol-lowed by spontaneous resolution over sev-eral months

Pathogenesis

Possible etiologic factors: sun exposure,trauma, human papilloma virus, geneticfactors, and immunosuppression


Solitary, firm, round, skin-colored or red-dish papule rapidly progressing to dome-shaped nodule, with a smooth shiny sur-face and a central keratinous plug; occurson sun-exposed areas of face, neck, anddorsum of the upper extremities; spontane-ous involution after many months


Squamous cell carcinoma; basal cell carci-noma; wart; seborrheic keratosis; invertedfollicular keratosis; atypical fibroxan-thoma; Merkel cell carcinoma; metastasis;sporotrichosis; coccidioidomycosis; NorthAmerican blastomycosis; prurigo nodularis

Therapy

Surgical excision

�

; radiation therapy;methotrexate 25 mg per ml intralesional,repeated every 2–3 weeks for up to5 treatments; fluorouracil 50 mg per ml int-ralesional, repeated every 2–3 weeks for upto 5 treatments

References

Schwartz RA (1994) Keratoacanthoma. Journal of the American Academy of Dermatology 30(1):1–19


Keratodermia 325

K

Keratoconjunctivitis sicca

�

Sjögren syndrome

Keratoderma

Synonym(s)

Keratodermia

Definition

Skin disorder consisting of a surface thatappears horny or scaly

References

Ratnavel RC, Griffiths WA (1997) The inherited palmoplantar keratodermas. British Journal of Dermatology 137(4):485–490

Keratoderma blennorrhagica

Definition

Hyperkeratotic and pustular condition ofthe palms and soles associated with Reiterdisease

References

Shupack JL, Stiller MJ, Haber RS (1991) Psoriasis and Reiter's syndrome. Clinics in Dermatology 9(1):53–58

Keratoderma hereditaria mutilans

�

Vohwinkel syndrome

Keratoderma palmoplangtaris diffusa with periodontosis

�

Papillon-Lefèvre syndrome

Keratoderma palmoplantaris striata

�

Striate keratoderma

Keratoderma palmoplantaris transgradiens

�

Mal de Meleda

Keratodermia

�

Keratoderma

Keratoderma.

Scaly plaques on the plantar aspects of the feet


326 Keratoelastoidosis

Keratoelastoidosis

�

Acrokeratoelastoidosis

Keratolysis plantaris sulcatum

�

Pitted keratolysis

Keratolytic winter erythema

Synonym(s)

Winter erythrokeratolysis

;

erythrokeratoly-sis hiemalis

;

Oudtshoorn skin

Definition

Form of ichthyosis characterized by cycli-cal erythema, hyperkeratosis, and recur-rent and intermittent peeling of the palmsand soles, particularly during winter

Pathogenesis

Unknown; autosomal dominant trait


Palmoplantar erythema with skin scaling;more pronounced in winter months


Erythrokeratodermia variabilis; progres-sive symmetric erythrokeratodermia; Gir-oux-Barbeau erythrokeratodermia withataxia; Greither disease; ichthyosis lineariscircumflexa; psoriasis; mycosis fungoides;lupus erythematosus; lamellar ichthyosis;gyrate erythema; atopic dermatitis

Therapy

Emollients

References

Danielsen AG, Weismann K, Thomsen HK (2001) Erythrokeratolysis hiemalis (keratolytic winter erythema): a case report from Denmark. Jour-nal of the European Academy of Dermatology & Venereology 15(3):255–256

Keratoma plantarum sulcatum

�

Pitted keratolysis

Keratomycosis nigricans palmaris

�

Tinea nigra

Keratosis diffusa fetalis

�

Ichthyosis fetalis

Keratosis follicularis

�

Darier disease

Keratosis follicularis et parafollicularis serpiginosa

�



Keratosis pilaris 327

K

Keratosis follicularis serpiginosa

�


Keratosis follicularis spinosa of Unna

�

Lichen spinulosus

Keratosis follicularis spinulosa

�

Lichen spinulosus

Keratosis, inverted follicular

�


Keratosis, lichenoid

�

Lichenoid keratosis

Keratosis palmaris et plantaris

Synonym(s)

Palmoplantar keratosis

;

palmoplantar kera-toderma

Definition

Heterogeneous group of disorders charac-terized by scaling and thickening of palmsand soles

References


Keratosis palmaris et plantaris with carcinoma of the esophagus

�

Tylosis

Keratosis palmo-plantaris circumscripta

� Tyrosinemia II

Keratosis pilaris

Synonym(s)Lichen pilaris; keratosis suprafollicularis;pityriasis pilaris

Keratosis pilaris. Acuminate, follicular papules on the cheek


328 Keratosis pilaris atrophicans

DefinitionDisorder of follicular keratinization, char-acterized by follicular keratotic papules

PathogenesisAutosomal dominant trait; arises fromexcessive accumulation of keratin at the fol-licular orifice

Clinical manifestationMultiple accuminate follicular keratoticpapules, sometimes with surounding ery-thema, most common on lateral arms,thighs and cheeks; association with ichthy-osis vulgaris and atopic dermatitis; worse indry climates and in the winter months;tends to improve with age

Differential diagnosisLichen spinulosus; folliculitis; milia; phryn-oderma; ichthyosis; pityriasis rubra pilaris;Darier disease; lichen planus

TherapyEmollients; tretinoin; alpha hydroxy acids;corticosteroids, topical, medium potency

ReferencesLateef A, Schwartz RA (1999) Keratosis pilaris.

Cutis 63(4):205–207


Synonym(s)Ulerythema ophryogenes; keratosis pilarisrubra atrophicans faciei; keratosis pilarisatrophicans faciei; folliculitis ulerythemareticulata; honeycomb atrophy; atropho-derma vermiculatum; ulerythema acnei-forme; atrophoderma reticulatum

DefinitionGroup of clinically related disorders charac-terized by follicular keratotic papules, vari-able perifollicular inflammation, and end-stage atrophy

PathogenesisUnknown; hereditary component

Clinical manifestationKeratosis pilaris rubra atrophicans faciei(ulerythema ophryogenes) variant: follicu-lar papules with erythematous halo, locatedover the lateral eyebrows; beginning shortlyafter birth and diminishing with ageAtrophoderma vermiculatum variant: onsetbetween age 5 and 12 years; follicular kera-totic papules with surrounding erythema;evolving into atrophic pits in a reticulatehoneycomb pattern

Differential diagnosisKeratosis pilaris; folliculitis; acne vulgaris;milia; pityriasis rubra pilaris

TherapyKeratolytics such as lactic acid 5% cream,urea 10% cream, or salicylic acid 2–5%cream or gel applied twice daily; alphahydroxy acids; isotretinoin

� Ulerythema ophryogenes

ReferencesFrosch PJ, Brumage MR, Schuster-Pavlovic C,

Bersch A (1988) Atrophoderma vermiculatum. Case reports and review. Journal of the Ameri-can Academy of Dermatology 18(3):538–542

Keratosis pilaris atrophicans faciei folliculitis ulerythema reticulata

� Keratosis pilaris atrophicans

Keratosis pilaris rubra atrophicans faciei



Ketoconazole 329

K

Keratosis rubra congenita

� Lamellar ichthyosis

Keratosis rubra figurata


Keratosis supracapitularis

� Knuckle pads

Keratosis suprafollicularis

� Keratosis pilaris

Keratosis-ichthyosis-deafness syndrome

� KID syndrome

Ketoconazole

Trade name(s)Nizoral



Mechanism of actionInhibition of fungal cell membrane ergos-terol synthesis



Common side effectsDermatologic: skin eruption, pruritusGastrointestinal: nausea and vomiting,diarrhea, abdominal painNeurologic: somnolence, dizziness, leth-argy, headache, nervousnessLaboratory: elevated liver enzymes

Serious side effectsDermatologic: anaphylaxisGastrointestinal: hepatic failure

Ketoconazole. Dermatologic indications and dosage







5–7 mg per kg PO daily for 6 months

Protothecosis 200–400 mg PO daily for 2–6 weeks Not established


200–400 mg PO dailyfor 6–12 months


Tinea versicolor 400 mg PO for 1 dose; repeat in 7 days

6.6 mg per kg PO for 1 dose; repeat in 7 days


330 KID syndrome

Endocrine: adrenal insufficiencyLaboratory: leukopenia, hemolytic anemia

Drug interactionsAmiodarone; amitriptyline; antacids; bar-biturates; buspirone; carbamazepine;cyclosporine; digoxin; glyburide/met-formin; H-2 blockers; protease inhibitors;phenytoin; pimozide; quinidine; rifampin;statins; sulfonylureas; tacrolimus; theophyl-line; vinca alkaloids; warfarin

Contraindications/precautionsHypersensitivity to drug class or compo-nent; achlorhydria; fungal meningitis; cau-tion in patients with hepatic insufficiencyor with use of other potentially hepatotoxicmedications

ReferencesRheney CC, Saddler CM (1998) Oral ketoconazole

in cutaneous fungal infections. Annals of Phar-macotherapy 32(6):709–711

KID syndrome

Synonym(s)Keratosis-ichthyosis-deafness syndrome

DefinitionDisorder characterized by keratitis, ichthy-osis-like keratoderma, and deafness

PathogenesisUnknown

Clinical manifestationVascularizing keratitis, with recurrent cor-neal ulcerations; congenital erythrokerato-derma; reticulated hyperkeratosis of thepalms and soles; sensorineural deafness;may develop chronic infections, scarringalopecia, squamous cell carcinoma, andneuromuscular disease

Differential diagnosisCongenital ichthyosiform erythroderma;lamellar ichthyosis; epidermolytic hyperk-eratosis; Netherton’s syndrome

TherapyEmollients; cyclosporine 2% ophthalmicsolution for keratitis

ReferencesLanger K, Konrad K, Wolff K (1990) Keratitis, ich-

thyosis and deafness (KID)-syndrome: report of three cases and a review of the literature. British Journal of Dermatology 122(5):689–697

Kikuchi’s disease


Kikuchi’s syndrome

Synonym(s)Kikuchi’s disease; Fujimoto’s disease;Kikuchi-Fujimoto disease; histiocyticnecrotizing lymphadenitis

DefinitionBenign, self-limited disorder characterizedby fever, chills, weight loss, and lymphaden-opathy

PathogenesisPossibly hypersensitivity reaction to infec-tious agent

Clinical manifestationPainless lymphadenopathy; mainly of thecervical lymph nodes; constitutional signsand symptoms: fever, chills, sore throat,myalgias; skin lesions including redplaques, facial erythema, crusted papulesand nodules, ulcerated papules; spontane-ous resolution in 1–4 months, with recur-rences


Kindler syndrome 331

K

Differential diagnosisViral exanthem; bacterial skin infection;mononucleosis; lupus erythematosus; lym-phoma; metastatic disease; sarcoidosis

TherapyNone

ReferencesYasukawa K, Matsumura T, Sato-Matsumura KC,

Takahashi T, Fujioka Y, Kobayashi H, Shimizu H (2001) Kikuchi's disease and the skin: case report and review of the literature. British Jour-nal of Dermatology 144(4):885–889

Kikuchi-Fujimoto disease


Kimura disease


Kimura’s disease

Synonym(s)Kimura disease; eosinophilic granuloma ofsoft tissue; eosinophilic hyperplastic lym-phogranuloma; eosinophilic lymphofollicu-losis; eosinophilic lymphofollicular granu-loma; eosinophilic lymphoid granuloma

DefinitionBenign, self-limited process, characterizedby subcutaneous tumors with a characteris-tic histologic appearance and lymphaden-opathy, and peripheral eosinophilia

PathogenesisAbnormal proliferation of lymphoid folli-cles and vascular endothelium; may repre-sent hypersensitivity reaction, perhaps to

arthropod bites, parasitic or candidal infec-tions

Clinical manifestationSolitary or multiple, firm, subcutaneousnodules, which usually are located on thehead or neck; lymphadonopathy; periph-eral eosinophilia

Differential diagnosisAngiolymphoid hyperplasia with eosi-nophilia; pyogenic granuloma; Kaposi’s sar-coma; eccrine cylindroma; Langerhans cellhistiocytosis; metastatic disease; Mikuliczdisease; parotid tumor

TherapySurgical excision�; triamcinolone 3–5 mgper ml intralesional; prednisone; radiationtherapy

ReferencesGumbs MA, Pai NB, Saraiya RJ, Rubinstein J,

Vythilingam L, Choi YJ (1999) Kimura's dis-ease: a case report and literature review. Jour-nal of Surgical Oncology 70(3):190–193

Kindler syndrome

Synonym(s)Kindler’s syndrome; poikiloderma of Kin-dler

DefinitionDisorder characterized by signs and symp-toms of both epidermolysis bullosa andpoikiloderma

PathogenesisUnknown

Clinical manifestationCongenital acral bullae; poikiloderma,beginning on sun-exposed skin and spread-ing to other areas over time; atrophy overthe hands and feet; gingivostomatitis


332 Kindler’s syndrome

Differential diagnosisRothmund-Thomson syndrome; hereditaryacrokeratotic poikiloderma of Weary; epi-dermolysis bullosa; Werner syndrome;Bloom’s syndrome

TherapyNone

ReferencesPatrizi A, Pauluzzi P, Neri I, Trevisan G, De Giorgi

LB, Pasquinelli G (1996) Kindler syndrome: re-port of a case with ultrastructural study and re-view of the literature. Pediatric Dermatology 13(5):397–402

Kindler’s syndrome

� Kindler syndrome

Kinky hair syndrome

� Menke’s kinky hair syndrome

Kitamura’s acropigmentatio reticularis

� Reticulate acropigmentation of Kitamura

Kitamura’s reticulate acropigmentation

� Reticulate Acropigmentation of Kitamura

Klein-Waardenburg syndrome

� Waardenburg syndrome

Klippel-Trenaunay syndrome

� Klippel-Trenaunay-Weber syndrome

Klippel-Trenaunay-Weber syndrome

Synonym(s)Klippel-Trenaunay syndrome; Angio-osteo-hypertrophy; nevus verrucosus osteohyper-trophicus syndrome; hemangiectasiahypertrophicans; nevus verucosus hyper-trophicans

DefinitionDisorder characterized by triad of port-wine stain, varicose veins, and bony andsoft tissue hypertrophy of an extremity

PathogenesisUnknown

Clinical manifestationMultiple port wine stains or other vascularnevi; hypertrophy of bones and soft tissuein the area of increased vascularity, mostcommonly in the lower limbs, the face andhead, or internal organs; occasional arterio-venous fistulas; varicose veins; occasionalsyndactyly and polydactyly, mental retarda-tion, and seizures

Differential diagnosisParkes-Weber syndrome; Mafucci syn-drome; proteus syndrome


Koilonychia 333

K

TherapyCompression garments; surgical removal ofvaricosities; flashlamp-pumped pulse dyelaser for port wine stain

ReferencesBlei F (2002) Vacular anomalies: From bedside to

bench and back again. Current Problems in Pediatric & Adolescent Health Care 32(3):72–93

Knuckle pads

Synonym(s)Halodermia; subcutaneous fibroma;keratosis supracapitularis; discrete kerato-derma

DefinitionAsymptomatic papules or nodules over theknuckles of the hands, often occurring afterrepetitive trauma to the area

PathogenesisOften of unknown cause; some cases asso-ciated with trauma, such as boxing or bit-ing of the knuckles in children; occasionalfamilial disorder

Clinical manifestationWell-circumscribed, flesh-colored papulesor nodules over the knuckles of the hands,most commonly over the proximal inter-phalangeal joint; may have erosion withfrictional trauma

Differential diagnosisAcanthosis nigricans; wart; granulomaannulare; callus; foreign body reaction;gouty tophus; osteoarthritis with Heberdennodules; rheumatoid nodule

TherapyProtective gloves or other form of paddingover the knuckles

ReferencesWon YH, Seo JJ, Kim SJ, Lee SC, Chun IK (1998)

Knuckle pad-like keratoderma: a new cutane-ous side reaction induced by tegafur. Interna-tional Journal of Dermatology 37(4):315–317

Koebner phenomenon

DefinitionAppearance of skin lesions of lichen pla-nus, warts, molluscum contagiosum, pso-riasis, or lichen nitidus along a site of injury

ReferencesWeiss G, Shemer A, Trau H (2002) The Koebner

phenomenon: review of the literature. Journal of the European Academy of Dermatology & Venereology 16(3):241–248

Kohlmeier-Degos syndrome

� Malignant atrophic papulosis

Koilonychia

DefinitionAbnormal shape of the fingernail where thenail plate has raised ridges and is thin andconcave

ReferencesGao XH, Li X, Zhao Y, Wang Y, Chen HD (2001)

Familial koilonychia. International Journal of Dermatology 40(4):290–291

Knuckle pads. Thickened skin over the knuckles, with erosions


334 Koplik’s spots

Koplik’s spots

DefinitionPunctate, white papules, often on an ery-thematous base, occurring on the buccalmucosa early in the course of rubeola

ReferencesRosa C (1998) Rubella and rubeola. Seminars in

Perinatology 22(4):318–322

Kraurosis vulvae

� Lichen sclerosus

Kunze riehm syndrome

� Michelin tire baby syndrome

Kwashiorkor

Synonym(s)None

DefinitionNutritional syndrome due to severe proteinmalnutrition with relative carbohydrateexcess

PathogenesisCaused by lack of essential amino acids,trace elements such as zinc, and vitamins inthe diet

Clinical manifestationFailure to thrive; edema; muscle wasting;retarded mental development; red, viola-ceous, and brown exfoliating plaques, giv-ing skin a “flaky paint” appearance; hairdry, lusterless, and light brown to gray incolor; dyschromia with hypopigmentationand hyperpigmentation; mucosal cheilosis

Differential diagnosisMarasmus; pellagra

TherapyIncrease in dietary animal protein

ReferencesLatham MC (1991) The dermatosis of kwash-

iorkor in young children. Seminars in Derma-tology 10(4):270–272

Kyrle disease

� Kyrle’s disease

Kyrle’s disease

Synonym(s)Kyrle disease; hyperkeratosis follicularis etparafollicularis in cutem penetrans

DefinitionPerforating disease associated with diabe-tes mellitus and renal failure, characterizedby formation of large papules with centralkeratin plugs

PathogenesisPossible contributing factors: metabolicderangements, mechanical trauma (e.g.,rubbing and scratching), or coiled-up hairswithin hyperkeratotic follicular lumina

Clinical manifestationSmall, scaly papule which enlarges to formred-brown papule or nodule with a centralkeratin plug; some follicular lesions;papules sometimes coalesce to form largerkeratotic plaques

Differential diagnosisReactive perforating collagenosis; perforat-ing folliculitis; elastosis perforans serpigi-nosa; prurigo nodularis; scabies; keratoa-canthoma; Darier disease; keratosis pilaris


Kyrle’s disease 335

K

TherapyTretinoin; isotretinoin; acitretin; vitamin A100,000 units PO daily for 30 days, repeatedafter a 1-month rest period

ReferencesHarman M, Aytekin S, Akdeniz S, Derici M (1998)

Kyrle's disease in diabetes mellitus and chronic renal failure. Journal of the European Academy of Dermatology & Venereology 11(1):87–88


L

L-tryptophan-induced eosinophilia-myalgia syndrome

�

Eosinophilia-myalgia syndrome

Labial lentigo

�

Lentigo

Lactic acid

�

Alpha hydroxy acid

Laffer-Ascher syndrome

�

Ascher’s syndrome

Lamellar ichthyosis

Synonym(s)

Nonbullous congenital ichthyosiformerythroderma

;

ichthyosis sebacea

;

ichthyosis congenita larva

;

keratosis rubra

Definition

Hereditary disorder of cornification, char-acterized by large, dark, plate-like scalesand underlying erythroderma

Pathogenesis

Autosomal recessive trait; mutation in thegene for transglutaminase 1(TGM1),enzyme involved in cornified cell envelopeformation


Neonate presents with tough, film-likemembrane that fissures when stretched(collodion membrane); membrane shed in10–14 days, leaving redness and scale, rang-ing from fine and white to thick, dark, andplate-like, arranged in a pattern resemblingfish skin; generalized pattern with accentu-ation in flexural areas such as the axilla,groin, antecubital fossa, and neck, whilesparing mucous membranes; scarring alo-pecia; nail dystrophy; ectropion; eclabium;

Lamellar ichthyosis.

Generalized erythema and scale in a neonate


338 Langerhans cell histiocytosis

conjunctivitis; small, deformed ears; inflex-ible digits due to taut skin


X-linked ichthyosis; congenital ichthyosi-form erythroderma; Conradi disease; Neth-erton syndrome; trichothiodystrophy;erythrodermic psoriasis; generalized sebor-rheic dermatitis; Rud syndrome; Sjögren-Larsson syndrome

Therapy

Emollients; alpha hydroxy acids; tretinoin;acitretin

References

Lacour M, Mehta-Nikhar B, Atherton DJ, Harper JI (1996) An appraisal of acitretin therapy in children with inherited disorders of keratiniza-tion. British Journal of Dermatology 134(6):1023–1029


Synonym(s)

Histiocytosis X

;

Langerhans cell granulo-matosis

;

type II histiocytosis

Definition

Group of disorders characterized by prolif-eration of bone-marrow-derived Langer-hans cells and mature eosinophils

Pathogenesis

Unclear whether disorders are neoplastic orinflammatory


Unifocal disease (eosinophilic granuloma):solitary bony lesion, usually asymptomaticMultifocal disease (Hand-Schuler-Chris-tian variant): diabetes insipidus; bonydefects; exophthalmos; other features: liver,spleen, lymph node infiltration; skinlesions, including noduloulcerative lesionsin the oral, perineal, perivulvar, or retroau-ricular regions

Acute disseminated disease (Letterer-Siwe):skin findings, including petechiae; scaly orcrusted yellow-brown papules, sometimescoalescing to form plaques, often in sebor-rheic distribution; exudative intertriginouslesions sometimes ulcerating; fever; ane-mia; thrombocytopenia; pulmonary infil-trates; lymphadenopathy; hepatosplenome-galy; neurologic involvement


Seborrheic dermatitis; dermatomyositis;mastocytosis; Wiskott-Aldrich syndrome;acrodermatitis enteropathica; Rosai-Dorf-man disease; xanthoma disseminatum; can-didiasis; listeriosis; herpes simplex virusinfection; varicella; infantile acropustulo-sis; leukemia; lymphoma; myeloma

Therapy

Localized skin involvement: high potencytopical corticosteroidsExtensive skin involvement: topical nitro-gen mustard; photochemotherapyMultisystem disease: chemotherapy

References

Zelger B, Burgdorf WH (2001) The cutaneous his-tiocytoses. Advances in Dermatology 17:77–114

Langerhans cell granulomatosis

�


Large plaque parapsoriasis

Synonym(s)


;

atrophic parapsori-asis

;

variegate dermatitis

;

poikilodermaatrophicans vasculare

;

poikiloderma vascu-lare atrophicans

;

lichenoid mycosis fun-goides


Laugier-Hunziger syndrome 339

L

Definition

Chronic, inflammatory skin disorder char-acterized by large scaly plaques

Pathogenesis

Unclear; may represent exaggerated hostresponse to chronic antigenic stimulation


Faint, salmon-colored plaques with arcuategeographic borders, often greater than 5 cmin diameter; may have an atrophic, ciga-rette, or tissue paper surface quality; lesionsappear on proximal extremities and trunkin a bathing trunk distribution; rare sponta-neous remission; sometimes progresses tocutaneous T-cell lymphoma


Small plaque parapsoriasis; psoriasis; seb-orrheic dermatitis; dermatophytosis; lupuserythematosus; lichen planus; pityriasisrosea; syphilis; mycosis fungoides; xerosis;nummular eczema

Therapy

Corticosteroids, topical, super potency;UVB phototherapy; photochemotherapy

References

Lambert WC, Everett MA (1981) The nosology of parapsoriasis. Journal of the American Acade-my of Dermatology 5(4):373–395

Larva currens

�

Strongyloidosis

Larva migrans

�


Late-onset prurigo of pregnancy

�


Lateral cervical cyst

�

Branchial cleft cyst

Latrodectism

�


Laugier disease

�



Synonym(s)

Laugier disease

;

essential melanotic pig-mentation

;

idiopathic lenticular mucocuta-neous pigmentation

Definition

Acquired, benign, macular hyperpigmenta-tion of the lips and buccal mucosa, associ-ated with longitudinal melanonychia andpigmentation elsewhere

Pathogenesis

Unknown


340 Launois-Bensaude syndrome


Solitary or confluent macular hyperpig-mentation of buccal mucosa, lips, gingiva,tongue, soft palate, and hard palate; pig-mentation also noted on neck, thorax,abdomen, dorsal and lateral aspects of fin-gers, soles, genitalia, perineum, perianalskin, and anal mucosa; nail pigmentationwithout dystrophy of fingers and toes; oneor two longitudinal bands per nail, whichtend to occur along the lateral aspects of thenail plate; half nail pigmentation or com-plete nail pigmentation


Nevus; melanoma; Addison’s disease;hemochromatosis; lichen planus; lupus ery-thematosus; amalgam tattoo; contactmucositis; drug-induced or chemical-induced hyperpigmentation; traumaticmelanonychia of the toenails; Peutz-Jegherssyndrome; physiologic melanoplakia andmelanonychia

Therapy

Frequency-doubled Q-switched Nd:YAGlaser, or HGM K1 krypton laser, or 532-nmdiode-pumped vanadate laser

References

Veraldi S, Cavicchini S, Benelli C, Gasparini G (1001) Laugier-Hunziker syndrome: a clinical, histopathologic, and ultrastructural study of four cases and review of the literature. Journal of the American Academy of Dermatology 25(4): 632–636

Launois-Bensaude syndrome

�


Lawrence syndrome

�


Ledderhose disease

�

Plantar fibromatosis

Leiomyoma

Synonym(s)

None

Definition

Benign soft-tissue neoplasm that arisesfrom smooth muscle

Pathogenesis

Unknown; three subtypes:Piloleiomyoma: arising from the arrectorpili muscle of the pilosebaceous unit.Angioleiomyoma: arising from smoothmuscle (i.e., tunica media) within the wallsof arteries and veins.Genital leiomyoma: derived from the dar-tos muscle of the scrotum or labia majora,or from the erectile muscle of the nipple


Piloleiomyoma: smooth, firm, tender, red-dish-brown papule or nodule; multiple pilo-leiomyomas sometimes occur on face,trunk, or extremities; grouped, der-matomal, or linear pattern; solitary pilolei-omyoma usually found on lower extremity;angioleiomyoma: well defined, deep der-mal papule or nodule which may be pain-ful; genital leiomyoma: found on vulva,scrotum, or nipple


Neurilemmoma; mastocytoma; dermatofi-broma; glomus tumor; neuroma; angiofi-broma; eccrine spiradenoma; breast carci-noma; plasmacytoma; leiomyosarcoma;neurofibroma

Therapy

Pain relief: nifedipine SR: 30–60 mg PO perday; phenoxybenzamine: 20–40 mg PO 2–


Lentigo 341

L

3 times per day; surgical excision of soli-tary tumor

References

Fearfield LA, Smith JR, Bunker CB, Staughton RC (2000) Association of multiple familial cutane-ous leiomyoma with a uterine symplastic leio-myoma. Clinical & Experimental Dermatology 25(1):44–47


Synonym(s)

Aleppo boil

;

Delhi boil

;

Baghdad boil

;

Biskra button

;

oriental sore

Definition

Protozoal parasitic disease spread by thebite of the sandfly

Pathogenesis

Protozoal promastigotes inoculated into thehost during the sandfly’s blood meal; pro-mastigotes enter macrophages, transformback into amastigotes, multiply, and spreadthroughout the reticuloendothelial system;helper T-cell subtype 1 (Th1) immuneresponse which induces disease resolution


Asymptomatic red papule which ulcerates;occurs at site of sandfly bite; heals overweeks to many months


Cutaneous tuberculosis; syphilis; leprosy;basal cell carcinoma; squamous cell carci-noma; deep fungal infection; pyodermagangrenosum

Therapy

Sodium antimony gluconate 20 mg per kgper day IV or IM for 20 days; ketoconazole600 mg PO daily for 4 weeks; hyperthermia

References

Hepburn NC (2001) Management of cutaneous leishmaniasis. Current Opinion in Infectious Diseases 14(2):151–154

Lentiginosis-deafness-cardiopathy syndrome

�

LEOPARD syndrome

Lentiginosis profusa syndrome

�

LEOPARD syndrome

Lentiginous hyperpigmentation

�

Nevoid hypermelanosis

Lentigo

Synonym(s)

Sun spot

;

liver spot

Definition

Small, sharply circumscribed, non-inflam-matory pigmented macule

Leishmaniasis, cutaneous.

Infiltrated, exudative, scaly, and crusted nodule on the knee


342 Lentigo maligna

Pathogenesis

Unknown; solar lentigo and ink-spot len-tigo associated with sun exposure in fair-skinned people; PUVA lentigo associatedwith photochemotherapy (PUVA); radia-tion lentigo caused by local high-dose irra-diation


Lentigo simplex: asymptomatic, round oroval, uniformly tan-brown to black macule,with jagged or smooth margins; lesions fewin number and occurring anywhere on skinor mucous membranesSolar lentigo: most commonly appearing onthe face, arms, dorsa of the hands, andupper part of the trunk; stellate-shaped,round or oval, uniformly tan-brown toblack macule; slowly increasing in numberand in size; lesions sometimes coalesce toform larger patchesInk spot lentigo: reticulated pattern, resem-bling spot of ink; limited to sun-exposedareas; single ink-spot lentigo among anextensive number of solar lentigines; PUVAlentigo: persistent, pale brown maculeappearing 6 months or longer after the startof PUVA therapy for psoriasis; resemblingsolar lentigo, but often with more irregularborders which may mimic ephelides; occur-rence closely associated with greater cumu-lative doses of PUVARadiation lentigo: resembles sun-inducedlentigo, but often has other histopathologicsigns of long-term cutaneous radiationdamage; considered an indicator of a priorexposure to a large single dose of ionizingradiationTanning-bed lentigo: usually occurs inwomen with history of tanning-bed use;similar to PUVA lentigoMucosal melanotic macule (labial lentigo;vulvar lentigo; penile lentigo): Labial lentigo almost always on the vermil-ion of the lower lip and usually solitary andasymptomatic; color ranges from brown toblue to blue-blackOral lentigo: appears on the gingiva, buccalmucosa, palate, and tonguePenile lentigo: most common sites: glanspenis, corona, coronal sulcus, and penile

shaft; varies in color from tan to brown todark brown, with irregular borders andskip areasVulvar lentigo: occurs anywhere on the gen-ital mucosa as a mottled, pigmented patchwith skip areas


Melanocytic nevus; lentigo maligna;melanoma; ephelides; actinic keratosis; seb-orrheic keratosis; traumatic tattoo; phyto-photodermatitis

Therapy

Frequency-doubled Q-switched Nd:YAGlaser, or HGM K1 krypton laser, or 532-nmdiode-pumped vanadate laser; hydroqui-none, with or without tretinoin

References

Schaffer JV, Bolognia JL (2000) The clinical spec-trum of pigmented lesions. Clinics in Plastic Surgery 27(3):391–408

Lentigo maligna

Synonym(s)

Hutchinson’s melanotic freckle

;

Hutchinson melanotic freckle

Definition

Intraepidermal melanocytic neoplasm,characterized by slow growth, on the face orother sun-exposed areas in fair-skinned,elderly individuals

Pathogenesis

Related to chronic, cumulative sun expo-sure


Most commonly affects the sun-exposedskin of the head and neck, with a predilec-tion for the nose and cheek; less commonsites: arm, leg, and trunk; conjunctivae andoral mucosa sometimes may be involved bycontiguous spread from cutaneous lesion;irregular mottling or flecking as lesion


LEOPARD syndrome 343

L

enlarges, with areas of dark brown or blackin some parts and lightening in others;lesion may be present for many years beforedermal invasion occurs


Melanocytic nevus, including atypicalmole; lentigo; seborrheic keratosis; pyo-genic granuloma; basal cell carcinoma;squamous cell carcinoma

Therapy

Surgical excision with 0.5 cm margin

�

; cry-otherapy; radiation therapy

References

Kaufmann R (2000) Surgical management of pri-mary melanoma. Clinical & Experimental Der-matology 25(6):476–481

Lentigo maligna melanoma

�

Melanoma

Lentigo senilis

�

Lentigo

Lentigo simplex

� Lentigo

LEOPARD syndrome

Synonym(s)Cardiocutaneous lentiginosis syndrome;multiple lentigines syndrome; generalizedlentiginosis; centrofacial lentiginosis;lentiginosis profusa syndrome; lentiginosis-

deafness-cardiopathy syndrome; cardio-cutaneous syndrome; progressive cardi-omyopathic lentiginosis

DefinitionAcronym depicting the main findings of asyndrome characterized by lentigines, elec-trocardiographic conduction abnormali-ties, ocular hypertelorism, pulmonary sten-osis, abnormalities of genitalia, retardationof growth, and deafness

PathogenesisPossible mutation in the stem cell pool ofthe neural crest in embryonic life

Clinical manifestationMany affected patients lack one or morecomponents of the defined syndrome;small, dark brown, polygonal, irregularlyshaped macules, often present on the face,neck, and upper part of the trunk, but alsoon palms, soles, and sclerae; axillary freck-ling; café au lait spots; localized hypopig-mentation; mild mental retardation; sen-sorineural hearing loss; short stature;mostly asymptomatic cardiac defects; dys-morphic face and/or skull; skeletal abnor-malities

Differential diagnosisAlbright syndrome; Carney’s syndrome;neurofibromatosis; Noonan syndrome;Peutz-Jeghers syndrome; nevi-atrialmyxoma-myxoid neurofibromata-ephe-lides (NAME or LAMB) syndrome

LEOPARD syndrome. Multiple brown macules on the forearm


344 Leprechaunism

TherapyCosmetically disfiguring lentigines – fre-quency-doubled Q-switched Nd:YAG laser,or HGM K1 krypton laser, or 532-nm diode-pumped vanadate laser; hydroquinone,with or without tretinoin

ReferencesJozwiak S, Schwartz RA; Janniger CK (1996)

LEOPARD syndrome (cardiocutaneous lentigi-nosis syndrome). Cutis 57(4):208–214

Leprechaunism

Synonym(s)Donohue syndrome

DefinitionHereditary disorder characterized by insu-lin resistance resulting in growth delays,abnormalities affecting the endocrine sys-tem, distinctive characteristics of the headand face, low birth weight, skin abnormali-ties, and enlargement of the breast and clit-oris in females and the penis in males

PathogenesisAutosomal recessive disorder; exact geneticdefect unknown

Clinical manifestationInsulin resistance; acanthosis nigricans; dif-fuse, increased skin pigmentation;decreased subcutaneous tissue; skin atro-phy; hirsutism; gingival hypertrophy; ich-thyosis; abnormal facies; short stature;abnormal genitalia

Differential diagnosisCutis laxa; lipoatrophy; dwarfism; progeria


ReferencesKosztolanyi G (1997) Leprechaunism/Donohue

syndrome/insulin receptor gene mutations: a syndrome delineation story from clinicopatho-

logical description to molecular understand-ing. European Journal of Pediatrics 156(4):253–255

Lepromatous leprosy

� Leprosy

Leprosy

Synonym(s)Hansen’s disease; Hansen disease

DefinitionChronic granulomatous disease, caused byMycobacterium leprae, which principallyaffects the skin and peripheral nervous sys-tem

PathogenesisCausative organism, M. leprae, an obligateintracellular acid-fast bacillus with abilityto enter nerves which are in cooler parts ofthe body; tissue damage depends on degreeto which cell-mediated immunityexpressed, the extent of bacillary spreadand multiplication, appearance of tissue-damaging immunologic complications (i.e.,lepra reactions), and the development ofnerve damage and its sequelae

Leprosy. Scaly plaques, digital tip erosions, and sclerosis of the hand


Leptospirosis 345

L

Clinical manifestationIndeterminate leprosy: one to a few hypop-igmented or erythematous macules, withintact sensationTuberculoid leprosy: skin lesions few innumber; well-defined, erythematous largeplaques, with elevated borders withatrophic center; arciform or annularplaques; found on the face, limbs, or else-where, but sparing intertriginous areas andthe scalp; alternate presentation: large,asymmetric, hypopigmented macule; bothtypes of lesions anesthetic, have localizedalopecia, and sometimes spontaneouslyresolve in a few years, leaving pigmentarydisturbances or scars; neural involvementcauses tender, thickened nerves with subse-quent loss of function; great auricular nerveand superficial peroneal nerves often prom-inentBorderline tuberculoid leprosy: similar totuberculoid form, but lesions smaller andmore numerous, nerves less enlarged, andless alopeciaBorderline leprosy: numerous, asymmet-ric, moderately anesthetic, red, irregularlyshaped plaques less well defined than thosein the tuberculoid type; regional adenopa-thy sometimes presentLepromatous leprosy: only infectious stage;early cutaneous lesions consisting mainly ofpale, small, diffuse, symmetric macules,which become infiltrated later, with littleloss of sensation; nerves not thickened andsweating normal; alopecia of lateral eye-brows, eyelashes, and trunk, but scalp hairintact; lepromatous infiltrations either dif-fuse nodules (lepromas) or plaques, whichresult in appearance of leonine facies;brawny lower extremity edema; neuriticlesions symmetric and slow to develop; eyeinvolvement causes pain; photophobia,decreased visual acuity, glaucoma, andblindness; testicular atrophy produces ste-rility and gynecomastia; lymphadenopathyand hepatomegaly result from organ infil-tration; stridor and hoarseness from laryn-geal involvement; nasal infiltration some-times produces a saddle-nose deformity;aseptic necrosis and osteomyelitis

Reactional state: lepra type I reaction: usu-ally affects patients with borderline dis-ease; downgrading reaction represents shifttoward the lepromatous pole before the ini-tiation of therapy; reversal reaction diseaseshift toward tuberculoid pole after the initi-ation of therapy; lepra type II reactions(erythema nodosum leprosum): immunecomplex–mediated reaction occurring inpatients with borderline lepromatous orpolar lepromatous disease; crops of painfulred papules, usually manifesting after a fewyears of therapy and resolving spontane-ously after about 5 years; associated fever,malaise, joint pain, nerve pain, iridocycli-tis, dactylitis, and orchitis; Lucio phenome-non: common in Mexico and Central Amer-ica; cutaneous hemorrhagic infarcts inpatients with diffuse lepromatous leprosy

Differential diagnosisVitiligo; post-inflammatory hypopigmenta-tion; lupus erythematosus; syphilis; sar-coidosis; tuberculosis; leishmaniasis; gran-uloma annulare; psoriasis

TherapyPaucibacillary disease: dapsone and rifamp-in�; multibacillary disease: dapsone indefi-nitely, rifampin and clofazimine 50 mg POper day for 3 years�; reactional states: pred-nisone; thalidomide

ReferencesRamos-e-Silva M, Rebello PF (2001) Leprosy. Rec-

ognition and treatment. American Journal of Clinical Dermatology 2(4):203–211001

Leptospirosis

Synonym(s)Autumnal fever; seven-day fever;swineherd's disease; swamp fever; mudfever; Fort Bragg fever; Weil disease;canicola fever; rice-field fever; cane-cutterfever; hemorrhagic jaundice; Stuttgart dis-ease


346 Lesch-Nyhan disease

DefinitionBacterial infection caused by one of severalspecies of spirochete of genus Leptospira,which can be found in fresh water contami-nated by animal urine

PathogenesisCaused by pathogenic spirochetes of thegenus Leptospira; organisms enter hostthrough abrasions in healthy skin, throughsodden and waterlogged skin, directlythrough intact mucus membranes or con-junctiva, through the nasal mucosa and cri-briform plate, or through the lungs; duringacute infection, organisms multiply in thesmall blood vessel endothelium, resultingin damage and vasculitis, the direct cause ofthe various clinical manifestations

Clinical manifestationAnicteric leptospirosis: self-limited diseasesimilar to a mild flu-like illness; icteric lept-ospirosis (Weil disease): severe illness withmultiple organ system involvement; skinchanges: warm and flushed; transientpetechial eruption that can involve the pal-ate; in severe disease, jaundice and pur-pura; conjunctival suffusion; myalgia; signsof meningitis, including neck stiffness andrigidity, delirium, and photophobia; liverenlargement and tenderness from hepatitis

Differential diagnosisEnteric fever; viral hepatitis; hantavirusinfection; rickettsial disease; encephalitis;typhoid fever; dengue fever; viral meningi-tis; malaria

TherapyMild disease: doxycycline; amoxicillin;erythromycin; severe disease: penicillin G20–24 million units IV per day, divided into4 doses for 5–10 days�

ReferencesVinetz JM (2001) Leptospirosis. Current Opinion

in Infectious Diseases 14(5):527–538

Lesch-Nyhan disease

� Lesch-Nyhan syndrome

Lesch-Nyhan syndrome

Synonym(s)Kelley-Seegmiller syndrome; Lesch-Nyhandisease

DefinitionGenetic disorder associated with overpro-duction of uric acid, neurological disabil-ity, and behavioral problems

PathogenesisMutations in the HPRT gene on theX chromosome lead to deficiency of hypox-anthine-guanine phosphoribosyl trans-ferase (HPRT), which plays a key role in therecycling of the purine bases, hypoxanthineand guanine, into the purine nucleotidepools; with absence of HPRT, purine basesnot salvaged, but degraded and excreted asuric acid; synthetic rate for purines acceler-ated markedly, to compensate for purineslost by the failure of the salvage process,resulting in overproduction of uric acid;pathogenesis of neurological and behavio-ral features unclear

Clinical manifestationGrowth retardation; impaired cognitivefunction; behavioral problems withattempts at self-injury, such as self-amputa-tions of the fingers, biting of the lips,tongue, or oral mucosa; marked hyperuri-cemia leading to nephrolithiasis

Differential diagnosisMental retardation; sociopathic behavior;cerebral palsy

TherapyControl of hyperuricemia: allopurinol300 mg PO per day�; behavior modifica-tion therapy


Leukoderma acquisita centrifugum 347

L

ReferencesJinnah HA, De Gregorio L, Harris JC, Nyhan WL,

O'Neill JP (2000) The spectrum of inherited mutations causing HPRT deficiency: 75 new cases and a review of 196 previously reported cases. Mutation Research 463(3):309–326

Lethal cutaneous and gastrointestinal arterial thrombosis


Lethal midline granuloma


Letterer-Siwe disease



Synonym(s)Allergic angiitis; small vessel vasculitis;allergic cutaneous vasculitis

DefinitionHistopathologic term used to denote asmall vessel vasculitis, occurring in a heter-ogeneous group of disorders

PathogenesisExact mechanism unclear; possibly involvesimmune complexes, other autoantibodiessuch as antineutrophil cytoplasmic anti-body (ANCA), other inflammatory media-tors, and local factors that affect endothe-

lial cells and other adhesion molecules;associated with medications, infections,foods and food additives, rheumatic dis-eases such as lupus erythematosus, and,rarely, malignant processes

Clinical manifestationAsymptomatic, pruritic or painful, palpa-ble purpuric papules, sometimes coalesc-ing into plaques and/or ulcerating; mostfrequently observed on the legs, but any sitepossible; some lesions begin as urticarialpapules; systemic manifestations of lung,gastrointestinal, renal, or rheumatologicinvolvement reflected in signs and symp-toms referable to those organs

Differential diagnosisSeptic vasculitis (e.g., meningococcemia,gonococcemia); Wegener’s granulomatosis;polyarteritis nodosa; erythema multi-forme; Churg-Strauss syndrome; choles-terol emboli; benign pigmented purpura;amyloidosis; Buerger disease; infectiveendocarditis; Rocky Mountain spottedfever; thrombotic thrombocytopenic pur-pura; urticaria; Waldenström hypergamma-globulinemia; idiopathic thrombocytope-nia purpura; or other causes of decreasedplatelets

TherapyColchicine; dapsone; prednisone

ReferencesStone JH, Calabrese LH, Hoffman GS, Pusey CD,

Hunder GG, Hellmann DB (2001) Vasculitis. A collection of pearls and myths. Rheumatic Dis-eases Clinics of North America 27(4):677–728

Leukoderma acquisita centrifugum

� Halo nevus


348 Leukodermia lenticular disseminata

Leukodermia lenticular disseminata


Leukopathia guttata et reticularis symmetrica


Lice

� Pediculosis

Lichen amyloidosis

Synonym(s)Primary localized cutaneous amyloidosis

DefinitionDisorder characterized by deposition ofamyloid fibrils in the skin, without evi-dence of deposition in internal organs

PathogenesisFibrils arise from degenerating keratinoc-ytes, probably secondary to chronic itchingand scratching

Clinical manifestationIntensely pruritic, flesh-colored or red-brown, hyperkeratotic papules, most com-monly seen on the pretibial surfaces butalso on the feet and thighs; macular vari-ant: irregular hyperpigmented patches overthe back or chest

Differential diagnosisPost-inflammatory hyperpigmentation;lichen simplex chronicus; mycosis fun-

goides; contact dermatitis; prurigo nodula-ris; lichen planus; lichenoid drug eruption;pretibial myxedema; necrobiosis lipoidica;acanthosis nigricans; ashy dermatosis

TherapyCorticosteroids, topical, super potent; UVBphototherapy; severe underlying atopic der-matitis: cyclosporine

ReferencesBehr FD, Levine N, Bangert J (2001) Lichen amy-

loidosis associated with atopic dermatitis: clin-ical resolution with cyclosporine. Archives of Dermatology 137(5):553–555

Lichen aureus



� Papular mucinosis

Lichen nitidus

Synonym(s)None

Lichen nitidus. Flat-topped, flesh-colored papules, coalescing into plaques on the hands


Lichen planus 349

L

DefinitionChronic skin eruption characterized byasymptomatic, small, flat-topped, skin-colored papules

PathogenesisUnknown

Clinical manifestationMultiple 1–3 mm, sharply demarcated, clus-tered, round or polygonal, flat-topped, skin-colored shiny papules, most commonly ontrunk, thighs, forearms, and genitalia;Koebner phenomenon sometimes occurs

Differential diagnosisLichen planus; flat warts; lichen spinulosus;lichen amyloidosis; keratosis pilaris; lichenstriatus; id reaction; sarcoidosis

TherapyCorticosteroids, topical, super potent; pho-tochemotherapy

ReferencesArizaga AT, Gaughan MD, Bang RH (2002) Gener-

alized lichen nitidus. Clinical & Experimental Dermatology 27(2):115–117

Lichen pigmentosus

� Ashy dermatosis

Lichen pilaris


Lichen pilaris seu spinulosus of Crocker

� Lichen spinulosus

Lichen planopilaris

� Lichen planus

Lichen planus

Synonym(s)Lichen rubor

DefinitionPruritic eruption characterized by viola-ceous, polygonal papules, with fine reticu-lated scale

PathogenesisUnknown; cell-mediated immune responseto unknown stimuli; associated withhepatitis C infection, chronic active hepati-tis, and primary biliary cirrhosis

Clinical manifestationPruritic, discrete or confluent, polygonalviolaceous papules, with fine white scale(Wickham’s stria); mucous membraneinvolvement with white or gray streaksforming a linear or reticular pattern on aviolaceous background, most commonly onthe buccal mucosa and tongue; genitalinvolvement with annular papules on the

Lichen planus. Violaceous, polygonal, flat-topped papules over the wrist


350 Lichen ruber planus cum pigmentatione

glans penis; vulvar involvement with reticu-late papules or erosions, with dyspareunia,burning sensation, pruritus and vulvar andurethral stenosis; nail plate thinning withlongitudinal grooving and ridging andoccasional destruction of nail plate withptyrigium formation; follicular and perifol-licular, violaceous, scaly, pruritic papuleson the scalp, sometimes progressing toatrophic cicatricial alopecia (lichen plano-pilaris)Hypertrophic variant: pruritic, thick, scaly,violaceous plaques, usually on the anteriorleg; atrophic variant: few lesions, often rep-resenting the resolution of annular orhypertrophic lesionsErosive variant: chronic, painful erosionson the mucosal surfaces; evolve from sitesof previous non-erosive diseaseActinic variant: nummular plaques with ahypopigmented zone surrounding a hyper-pigmented center

Differential diagnosisPsoriasis; pityriasis rosea; lupus erythema-tosus; lichenoid drug eruption; scabies;graft versus host disease; lichen simplexchronicus; lichen nitidus; syphilis; pemphi-gus foliaceus; squamous cell carcinoma ofthe oral mucosa

TherapyCorticosteroids, topical, super potent;�;severe, generalized disease – prednisone;acitretin; isotretinoin; photochemotherapy

ReferencesCapella GL, Finzi AF (2000) Psoriasis, lichen pla-

nus, and disorders of keratinization: unap-proved treatments or indications. Clinics in Dermatology 18(2):159–169

Lichen ruber planus cum pigmentatione


Lichen rubor

� Lichen planus

Lichen sclerosus

Synonym(s)Lichen sclerosus et atrophicus; kraurosisvulvae; balanitis xerotica obliterans

DefinitionChronic inflammatory dermatosis resultingin white plaques with epidermal atrophy

PathogenesisUnknown; inflammation and abnormalfibroblast function in the upper dermiscausing fibrosis of the upper dermis

Clinical manifestationAsymptomatic or slightly pruritic, white,polygonal papules coalescing into shinyplaques, often with follicular prominenceand occasional isomophic response (Koeb-ner phenomenon); vulvar variant (krauro-sis vulvae): often intense pruritus; gradualobliteration of the labia minora and steno-sis of the introitus; occasional vesicles orhemorrhagic bullae; hourglass, butterflypattern involving perivaginal and perianal

Lichen sclerosus. Hypopigmented, sclerotic plaques, with effacement of the labia minora


Lichen simplex chronicus 351

L

areas; male genital variant (balanitis xerot-ica obliterans): usually confined to glanspenis and prepuce or foreskin remnants;sometimes causes phimosis after extensivesclerosis of prepuce

Differential diagnosisMorphea; scleroderma; child abuse; lichenplanus; psoriasis; tinea versicolor; vitiligo;idiopathic guttate hypomelanosis; post-inflammatory hypopigmentation; aneto-derma; Bowen’s disease

TherapyGenital disease: corticosteroids, topical,super potent�; tretinoin; acitretin;isotretinoin; extragenital disease: no effec-tive therapy

ReferencesNeill SM, Ridley CM (2001) Management of ano-

genital lichen sclerosus. Clinical & Experimen-tal Dermatology 26(8):637–643

Lichen sclerosus et atrophicus

� Lichen sclerosus

Lichen sclerosus et atrophicus of the penis

� Balanitis xerotica obliterans

Lichen sclerosus of the penis


Lichen scrofulosorum



Synonym(s)Neurodermatitis circumscripta; circum-scribed neurodermatitis; lichen simplexchronicus of Vidal

DefinitionLichenification of the skin with variablescale, due to repetitive scratching or rub-bing

PathogenesisUnknown; occurs more frequently in thosewith atopic diathesis; psychological factorsoperative in some cases; caused by chronicrubbing or scratching

Clinical manifestationOne or more slightly erythematous, scaly,well-demarcated, lichenified, firm plaques,often with hyperpigmentation; most com-mon locations: posterior neck, scalp, exten-sor aspect of extremities, vulva in women,and scrotum in men

Lichen simplex chronicus. Irregular eroded nodule with surrounding lichenification


352 Lichen simplex chronicus of Vidal

Prurigo nodularis variant: discrete, firm,purpuric nodules or papules, often withoverlying erosion; occurs on extensor sur-faces of arms and legs, posterior neck,upper back and trunk

Differential diagnosisAcanthosis nigricans; lichen amyloidosis;insect bite reaction; psoriasis; contact der-matitis; lupus erythematosus; dermatophy-tosis; stasis dermatitis; nummular eczema;lichen planus; acne keloidalis; atopic der-matitis

TherapyCorticosteroids, topical, high potency, orcorticosteroids, topical, super potency�;triamcinolone 3–5 mg per ml intralesional;antihistamines, first generation

ReferencesJones RO (1996) Lichen simplex chronicus. Clin-

ics in Podiatric Medicine & Surgery 13(1):47–54

Lichen simplex chronicus of Vidal


Lichen spinulosus

Synonym(s)Keratosis follicularis spinulosa; lichenpilaris seu spinulosus of Crocker; keratosisfollicularis spinosa of Unna

DefinitionDisorder characterized by plaques consist-ing of follicular keratotic papules

PathogenesisUnknown

Clinical manifestationSymmetrical, accuminate, keratoticpapules, affecting the neck, buttocks, abdo-men, trochanters, knees, and extensor sur-faces of the arms; may coalesce into plaques

Differential diagnosisLichen nitidus; lichen simplex chronicus;keratosis pilaris; phrynoderma; flat warts;lichen planopilaris; pityriasis rubra pilaris;Darier disease


ReferencesStrickling WA, Norton SA (2000) Spiny eruption

on the neck. Diagnosis: Lichen spinulosus (LS). Archives of Dermatology 136(9):1165–1170

Lichen striatus

Synonym(s)Linear lichenoid dermatosis; linear neuro-dermatitis; blaschkitis; Blaschko linearacquired inflammatory skin eruption; zonaldermatosis; linear dermatosis; systematizedlichenification; linear eczema

DefinitionInflammatory papular eruption with a dis-tinctive linear distribution, often followingBlaschko’s lines

PathogenesisUnknown

Clinical manifestationMost common on extremities, trunk, andneck; flat- topped, erythematous or skin-colored, lichenoid, scaly papules, coalesc-ing into small plaques in a continuous orinterrupted linear band; spontaneous reso-lution in months to 1 year, often in the sameproximal to distal fashion in which theyappeared, leaving variable dyspigmentation


Lichenoid mycosis fungoides 353

L

Differential diagnosisInflammatory linear verrucous epidermalnevus; lichen planus; atopic dermatitis;lichen simplex chronicus; Darier disease;wart; porokeratosis

TherapyCorticosteroids, topical, high potency;emollients

ReferencesHauber K, Rose C, Brocker EB, Hamm H (2000)

Lichen striatus: clinical features and follow-up in 12 patients. European Journal of Dermatolo-gy 10(7):536–539

Lichen tropicus

� Miliaria

Lichenoid benign keratosis


Lichenoid chronic dermatosis


Lichenoid keratosis

Synonym(s)Benign lichenoid keratosis; solitary lichenplanus; solitary lichen planus-like kerato-sis; lichenoid benign keratosis

DefinitionSolitary, benign skin lesion with the histo-logic features of a lichenoid reaction

PathogenesisUnclear; may be inflammatory stage of len-tigo or seborrheic keratosis

Clinical manifestationSharply demarcated, scaly, red-brown,almost flat papule, often on sun-exposedskin of the extremities

Differential diagnosisLentigo; seborrheic keratosis; lichen pla-nus; lichenoid drug eruption; lupus ery-thematosus; wart; Bowen’s disease; superfi-cial basal cell carcinoma

TherapyDestruction by liquid nitrogen cryotherapyor electrodesiccation and curettage

ReferencesJang KA, Kim SH, Choi JH, Sung KJ, Moon KC,

Koh JK (2000) Lichenoid keratosis: a clinico-pathologic study of 17 patients. Journal of the American Academy of Dermatology 43(3):511–516

Lichenoid mycosis fungoides


Lichenoid keratosis. Sharply demarcated, reddish-brown verrucous papule on the leg


354 Lichenoid pigmented purpura of Gougerot and Blum

Lichenoid pigmented purpura of Gougerot and Blum


Light-sensitive seborrheid


Lindane

Trade name(s)None


Drug classOrganochloride anti-parasitic agent

Mechanism of actionBlocks neural transmission

Dosage form1% lotion; 1% shamopoo


Common side effectsCutaneous: irritant dermatitisNeurologic: dizziness, anxiety, CNS stimula-tion

Serious side effectsNeurologic: neurotoxicity; seizures


Contraindications/precautionsHypersensitivity to drug class or compo-nent; pregnancy; lactating mother

ReferencesWendel K, Rompalo A (2002) Scabies and pedicu-

losis pubis: an update of treatment regimens and general review. Clinical Infectious Diseases 35(Suppl 2):S146–51

Linea alba (bite line)

DefinitionThin, white line running from angle ofmouth to pterygomandibular raphae,caused by pressure of cheek into line ofocclusion

ReferencesLaude TA (1995) Approach to dermatologic disor-

ders in black children. Seminars in Dermatolo-gy 14(1):15–20

Lindane. Dermatologic indications and dosage


Pediculosis capitis Apply shampoo for 30 minutes > 2 years old – apply shampoo for 30 minutes

Pediculosis pubis Apply shampoo for 30 minutes > 2 years old – apply shampoo for 30 minutes

Scabies Apply lotion to whole body except for the head; reapply in 7 days

> 2 years old – apply lotion to whole body except for the head; reapply in 7 days


Linear neurodermatitis 355

L

Linear and whorled nevoid hypermelanosis

� Nevoid hypermelanosis

Linear dermatosis

� Lichen striatus

Linear eczema

� Lichen striatus



Linear IgA bullous disease


Linear IgA dermatosis

Synonym(s)Linear IgA bullous dermatosis; linear IgAbullous disease; chronic bullous disease ofchildhood

DefinitionAutoimmune, subepidermal, vesiculobul-lous disease with IgA immunoreactantsdeposited in the skin

PathogenesisAntibody to 97 kDa protein in the base-ment membrane zone causes complementactivation and neutrophil chemotaxis; lossof adhesion at the dermal-epidermal junc-tion produces blisters; 97 kDa protein mayrepresent a portion of the extracellulardomain of the 180 kDa bullous pemphigoidantigen

Clinical manifestationClear and/or hemorrhagic vesicles or bul-lae on normal, erythematous, or urticarialskin; may also have erythematous plaques,blanching macules and papules, or ery-thema multiforme-like lesions; oral mucousmembrane lesions, including red patches,vesicles, ulcerations, erosions, desquama-tive gingivitis, or cheilitis; possibly medica-tion related in some cases, most commonlywith vancomycin

Differential diagnosisBullous pemphigoid; erythema multi-forme; epidermolysis bullosa; epidermoly-sis bullosa acquisita; dermatitis herpeti-formis; impetigo; pemphigus foliaceus;pemphigus vulgaris; herpes simplex virusinfection; herpes zoster

TherapyDapsone; prednisone; tetracycline; niacina-mide

ReferencesRabinowitz LG, Esterly NB (1993) Inflammatory

bullous diseases in children. Dermatologic Clinics 11(3):565–581

Linear lichenoid dermatosis

� Lichen striatus

Linear neurodermatitis

� Lichen striatus


356 Linear porokeratosis

Linear porokeratosis

� Porokeratosis

Lingua nigra

� Hairy tongue

Lingua plicata

Synonym(s)Fissured tongue; scrotal tongue; furrowedtongue

DefinitionCondition characterized by grooves vary-ing in depth and noted along the dorsal andlateral aspects of the tongue

PathogenesisPossibly polygenic or autosomal inherit-ance pattern

Clinical manifestationAsymptomatic fissured tongue, affectingthe dorsum and often extending to the lat-eral borders; fissures or grooves sometimesinterconnected, artificially separating thedorsum of the tongue into lobules

Differential diagnosisGeographic tongue; cheilitis granulomatosa

TherapyNo therapy if asymptomatic; brushing oftongue with toothbrush to remove embed-ded debris

ReferencesFisher BK. Linzon CD (1997) Scrotal glans penis

(glans penis plicatum) associated with scrotal tongue (lingua plicata). International Journal of Dermatology 36(10):762–763997

Lingua villosa

� Hairy tongue

Lingua villosa nigra

� Hairy tongue

Lipid histiocytosis


Lipoatrophy


Lipodermoid

� Dermoid cyst

Lipoglycoproteinosis


Lipogranulomatosis subcutanea



Lipoma 357

L

Lipoid dermatoarthritis

� Multicentric reticulohistiocytosis

Lipoid proteinosis

Synonym(s)Hyalinosis cutis et mucosae, Urbach-Wiethe disease; lipoproteinosis;lipoglycoproteinosis; lipoidosis cutis etmucosae

DefinitionHereditary disease characterized by deposi-tion of eosinophilic hyaline-like material inthe skin, larynx, mucous membranes, brain,and other internal organs

PathogenesisAutosomal recessive trait; unclear whetherdeposit of eosinophilic material in organs isprimary or secondary phenomenon; possi-bly an abnormality of collagen metabolismor a lysosomal disease

Clinical manifestationHoarseness in infancy; presents early in lifewith recurrent vesicles, bullae, and hemor-rhagic crusts, particularly on the face, onmucous membranes, and on distal extremi-ties, which heal with ice-pick scarring; laterin life, skin becomes waxy, thickened, andyellow; papules, plaques, and nodules onthe face, axillae, and scrotum; verrucouslesions on the elbows, knees, and sites oftrauma; beaded papules along the eyelidmargins (moniliform blepharitis); patchyalopecia where hyaline deposits are present;cobblestone appearance with multiplepapules on the tongue, lips, and gingiva;tongue may have woody induration andulceration; transient swelling of the lips andtongue; abnormal dentition; involvement oflarynx and vocal cords sometimes causesrespiratory distress; bilateral temporal lobecalcifications sometimes lead to seizures

Differential diagnosisAmyloidosis; papular mucinosis; xantho-mas; colloid milia; myxedema; erythropo-etic protoporphyria

TherapyAcitretin; dermabrasion; surgical resectionof vocal cord papules


diseases. Part I. Journal of the American Acad-emy of Dermatology 39(2 Pt 1):149–171

Lipoid rheumatism

� Multicentric reticulohistiocytosis

Lipoidosis cutis et mucosae


Lipoma

Synonym(s)Fatty tumor

DefinitionBenign tumor of fat cells, presenting as sub-cutaneous nodules

PathogenesisUnknown; differs biochemically from nor-mal fat by increased lipoprotein lipase lev-els and larger number of precursor cells

Clinical manifestationAsymptomatic, slow-growing, soft, subcu-taneous nodule, most commonly over theback, neck, shoulders, and proximal upperextremities


358 Lipomatosis

Differential diagnosisEpidermoid cyst; liposarcoma; panniculi-tis; neurofibroma; leiomyoma; blue rubberbleb nevus syndrome; glomus tumor

TherapySurgical excision; liposuction

ReferencesSalam GA (2002) Lipoma excision. American

Family Physician. 65(5):901–904

Lipomatosis

� Lipoma

Lipophagic panniculitis of childhood


Lipoproteinosis


Liposarcoma

Synonym(s)Atypical lipoma; atypical lipomatoustumors; malignancy of fat cells

DefinitionMalignancy of fat cells

PathogenesisTrauma possibly a co-factor in some cases

Clinical manifestationAsymptomatic, exophytic, slow-growing,dome-shaped or polypoid tumor

Differential diagnosisLipoma; neurofibroma; dermatofibrosar-coma; angiofibroma; rhabdomyosarcoma;leiomyosarcoma; fibrous histiocytoma lipo-blastoma in infants and children


ReferencesWong CK, Edwards AT, Rees BI (1997) Liposarco-

ma: a review of current diagnosis and manage-ment. British Journal of Hospital Medicine 58(11):589–591

Livedo reticularis

DefinitionMottling of the skin, usually on the legs



Livedo reticularis with summer/winter ulcerations

� Livedoid vasculitis

Livedo reticularis. Reticulated red-brown patches on the lower extremities


Loose anagen hair of childhood 359

L

Livedo vasculitis


Livedoid vasculitis

Synonym(s)Livedo vasculitis; livedo reticularis withsummer/winter ulcerations; segmentalhyalinizing vasculitis

DefinitionChronic vasculopathy characterized byrecurrent lower extremity ulcerations thatheal with stellate white scars

PathogenesisUnknown; deposition of fibrinoid materialin dermal vessels causes overlying ischemicchange, suggesting occlusive or thromboticmechanism

Clinical manifestationSmall, painful, purpuric macules andpapules that ulcerate and heal with stellatewhite atrophic scars, with surrounding tel-angiectasias and hyperpigmentation; sea-sonal course, with outbreaks in the sum-mer and winter; occurs more often inwomen

Differential diagnosisLivedo reticularis (retiform purpura);hypersensitivity vasculitis; stasis ulcera-tion; cholesterol emboli; septic emboli;anti-phospholipid antibody syndrome;lupus erythematosus

TherapyAntiplatelet therapy, such as aspirin anddipyridamole; fibrinolytic agents, such astissue plasminogen activator or danazol;anticoagulants, such as warfarin; anti-inflammatory agents, such as prednisone ornon-steroidal anti-inflammatory agents;pentoxifylline

ReferencesFogelman JP (2001) Livedoid vasculitis. Derma-

tology Online Journal 7(1):19

Liver spot

� Lentigo

Loa loa

� Filariasis

Lobular capillary hemangioma


Localized scleroderma

� Morphea

Loiasis

� Filariasis

Loose anagen hair of childhood

� Loose anagen hair syndrome


360 Loose anagen hair syndrome

Loose anagen hair syndrome

Synonym(s)Loose anagen syndrome; short anagen syn-drome; loose anagen hair of childhood

DefinitionDisorder characterized by anagen hairswith abnormal morphology, easily andpainlessly pulled or plucked from the scalp,leading to short, abnormal-appearing hair

PathogenesisUnknown; abnormal keratinization of theinner root sheath may be part of the patho-logical process

Clinical manifestationSparse growth of thin, fine hair and diffuseor patchy alopecia without inflammation orscarring; hair easily pulled out and unman-ageable, lusterless, dry, dull, or matted; hairgrows only to relatively short length

Differential diagnosisAlopecia areata; tinea capitis; traction alo-pecia; trichotillomania; telogen effluvium;lichen planopilaris; thyroid disease; irondeficiency anemia; anagen effluvium

TherapyNone

ReferencesLi VW, Baden HP, Kvedar JC (1996) Loose anagen

syndrome and loose anagen hair. Dermatologic Clinics 14(4):745–751

Loose anagen syndrome


Loratadine

� Antihistamines, second generation

Loss of hair, partial or complete

� Alopecia

Louis-Bar syndrome

� Ataxia-telangiectasia

Louse-borne relapsing fever

� Relapsing fever

Louse borne typhus

� Epidemic typhus

Loxoscelism

� Brown recluse spider bite

Lues

� Syphilis


Lupus erythematosus, discoid 361

L

Lupus anticoagulant syndrome



Synonym(s)Acute lupus erythematosus

DefinitionHeterogeneous connective tissue diseaseassociated with polyclonal B-cell activationand multisystem involvement

PathogenesisUnclear; interplay of genetic, environmen-tal, and hormonal factors; association withhuman leukocyte antigen DR2 and humanleukocyte antigen DR3; ultraviolet light actsas trigger; certain viruses may be co-factors

Clinical manifestationConfluent erythema and edema, most com-monly over malar eminence and nasalbridge (butterfly eruption); vesicles andbullae, often over lower extremities; mor-billiform eruption in a sunlight distribu-tion; other sites of involvement: forehead,periorbital area, and sides of the neck;superficial ulceration, primarily involvingthe posterior surface of the hard palate

Differential diagnosisRosacea; tinea faciei; seborrheic dermatitis;polymorphous light eruption; erythemamultiforme; phototoxic drug eruption;solar urticaria; dermatomyositis

TherapyPrednisone�; azathioprine; cyclophospha-mide; thalidomide; hydroxychloroquine;intravenous IgG (IVIG): 0.5–1 g per kg perday for 4 days

ReferencesCallen JP (2002) Management of skin disease in

patients with lupus erythematosus. Best Prac-tice & Research in Clinical Rheumatology 16(2):245–264


Synonym(s)Chronic cutaneous lupus erythematosus;discoid lupus erythematosus

DefinitionChronic, scarring, atrophy-producing, pho-tosensitive dermatosis, sometimes occur-ring in patients with systemic lupus ery-thematosus

PathogenesisPossible genetic predisposition; triggeredby ultraviolet light

Clinical manifestationMinimally scaly, erythematous papule orplaque, evolving with hypopigmentation inthe central area and hyperpigmentation atthe active border, often starting after sunexposure; as lesion evolves, dilation of fol-licular openings occurs with keratinousplug (follicular plugging; patulous folli-cles); resolution with atrophy and scarring;localized variant: head and neck affected;usually only a few lesions; widespread vari-

Lupus erythematosus, discoid. Multiple, red eroded papules on the face and lip


362 Lupus erythematosus panniculitis

ant: areas other than head and neckaffected; more likely to develop systemiclupus erythematosus

Differential diagnosisLichen planus; actinic keratosis; granulomafaciale; Jessner lymphocytic infiltration ofthe skin; granuloma annulare; sarcoidosis;dermatomyositis; rosacea; tinea faciei;squamous cell carcinoma

TherapyCorticosteroids, topical, super potent; tri-amcinolone 3–4 mg per ml intralesional;hydroxychloroquine; prednisone; thalido-mide

ReferencesCallen JP (1994) Treatment of cutaneous lesions in

patients with lupus erythematosus. Dermato-logic Clinics 12(1):201–206

Lupus erythematosus panniculitis

� Lupus panniculitis


Synonym(s)Subacute cutaneous lupus erythematosus

DefinitionNonscarring, photosensitive dermatosisthat may occur in patients with systemiclupus erythematosus, Sjögren syndrome,deficiency of the second component ofcomplement, or after exposure to certainmedications

PathogenesisGenetic predisposition; strong associationwith anti-Ro (SS-A) autoantibodies; ultravi-

olet light modulation of autoantigens, epi-dermal cytokines, and adhesion molecules,with keratinocyte injury and apoptosis

Clinical manifestationBegins as a minimally scaly, erythematouspapule or a small plaque, in sun-exposeddistributionPapulosquamous variant: mimics psoriasisor lichen planusAnnular variant: similar to erythema annu-lare centrifugumNeonatal variant: transient infiltrated redpapules and plaques on the face; usuallyresolves by age 4–6 months; some patientswith congenital heart block requires pace-makerSystemic signs and symptoms: Sjögren syn-drome, fatigue, arthritis, pleuritis or peri-carditis; several drugs capable of produc-ing this syndrome, most commonly thi-azide diuretics

Differential diagnosisPsoriasis; erythema annulare centrifugum;erythema multiforme; tinea corporis;lichen planus; sarcoidosis; granulomaannulare; Lyme disease; dermatomyositis;hypersensitivity vasculitis; polymorphouslight eruption

TherapyHydroxychloroquine�; corticosteroids, top-ical, super potent; prednisone; thalidomide

ReferencesMcCauliffe DP (2001) Cutaneous lupus erythema-

tosus. Seminars in Cutaneous Medicine & Sur-gery 20(1):14–26

Lupus miliaris disseminatus faciei

� Rosacea


Lyme disease 363

L

Lupus panniculitis

Synonym(s)Lupus profundus; lupus erythematosuspanniculitis

DefinitionVariant of chronic cutaneous lupus ery-thematosus, characterized by subcutane-ous nodules and atrophy

PathogenesisUnknown

Clinical manifestationMultiple, discrete, firm, subcutaneous nod-ules, with evolution into atrophic papulesor nodules; sometimes associated withlesions of discoid lupus erythematosus;occasionally occurs in patients with sys-temic lupus erythematosus

Differential diagnosisErythema nodosum; erythema induratum(nodular vasculitis); superficial throm-bophlebitis; Weber-Christian disease; pan-creatic panniculitis; inflamed epidermoidcyst; atrophoderma of Pasini and Pierini;morphea

TherapyHydroxychloroquine�; surgical excision

ReferencesPeters MS, Su WP (1989) Lupus erythematosus

panniculitis. Medical Clinics of North America 73(5):1113–1126

Lupus profundus

� Lupus panniculitis

Lupus vulgaris


Lutz mycosis


Lyell syndrome


Lyme borreliosis

� Lyme disease

Lyme borreliosis, late phase

� Acrodermatitis chronica atrophicans

Lyme disease

Synonym(s)Lyme borreliosis

DefinitionSystemic infection caused by the spirocheteBorrelia burgdorferi, after inoculation intothe skin by a tick bite


364 Lymphadenosis benigna cutis

PathogenesisSpriochetes introduced into the skin by abite from an infected Ixodes tick; three tickspecies: B burgdorferi sensu stricto strainconstituting all North American isolates;B garinii found exclusively in Europe;B afzelii most common organism causingacrodermatitis chronica atrophicans

Clinical manifestationEarly Lyme disease: sometimes presentswith flulike illness; erythema migrans:begins as an erythematous macule orpapule at the site of tick bite, often withcentral punctum at the site of the bite; erup-tion gradually expands with central clear-ing over days to weeks; multiple lesions mayoccur; borrelial lymphocytoma: bluish rednodules, usually on earlobe or nipple; acro-dermatitis chronica atrophicans: begins asan inflammatory phase marked with edemaand erythema, usually on the distal extrem-ities; lesions on posterior heels and dorsalsurfaces of the hands, feet, elbows, andknees; gradual central progression overmonths to years; systemic involvement,including Bell’s palsy, arthritis; chronicfatigue syndrome; meningoradiculoneuri-tis (Bannwarth syndrome); carditis; andchronic meningoencephalitis

Differential diagnosisErythema marginatum rheumaticum; ery-thema gyratum repens; urticaria; granu-loma annulare; sarcoidosis; tinea corporis;

seborrheic dermatitis; lupus erythemato-sus; benign lymphocytic infiltrate; rheuma-toid arthritis; psoriatic arthritis; lupus ery-thematosus; Reiter syndrome; gonococcalarthritis

TherapyDoxycycline; amoxicillin; erythromycin

ReferencesRavishankar J, Lutwick LI (2001) Current and fu-

ture treatment of Lyme disease. Expert Opin-ion on Pharmacotherapy 2(2):241–251

Lymphadenosis benigna cutis

� Pseudolymphoma

Lymphangioma

Synonym(s)Cutaneous lymphangioma; lymphangiomacircumscriptum; cavernous lymphangi-oma; cystic hygroma

DefinitionCongenital hamartomatous malformationof the lymphatic system, involving the skinand subcutaneous tissues

PathogenesisCisterns arising from primitive lymph sacfailing to connect with the rest of the lym-phatic system during embryonic develop-ment; contractions increase the intramuralpressure, causing dilated channels to pro-trude from the walls of the cisterns towardthe skin; vesicles seen in lymphangiomacircumscriptum represent outpouchings ofthese dilated vessels

Lyme disease. Large, red plaque with an active advancing margin


Lymphogranuloma venereum 365

L

Clinical manifestationLymphangioma circumscriptum: smallclusters of vesicles, varying in color frompink to red to black (secondary to hemor-rhage); sometimes have verrucous surfaceCavernous lymphangioma: rubbery, multi-lobulated subcutaneous nodulesCystic hygroma: large, soft, and translucentcystic lesion, occurring in the neck, axilla,and parotid area

Differential diagnosisHerpes simplex virus infection; herpeszoster; wart; epidermoid cyst; lipoma;hemangioma; neurofibroma; epidermalnevus; melanoma; lymphangiectasia; bran-chiogenic cyst; thyroglossal duct cyst

TherapyComplete surgical excision�; laser abla-tion; cryotherapy, sclerotherapy; electro-cautery

ReferencesMulliken JB, Fishman SJ, Burrows PE (2000) Vas-

cular anomalies. Current Problems in Surgery 37(8):517–584

Lymphangioma circumscriptum

� Lymphangioma

Lymphangiosarcoma of Stewart-Treves

� Stewart-Treves syndrome

Lymphatic filariasis

� Filariasis

Lymphocytic infiltrate of Jessner

� Jessner lymphocytic infiltration of skin

Lymphocytic infiltration of skin

� Jessner’s lymphocytic infiltration of skin

Lymphocytoma cutis

� Pseudolymphoma

Lymphogranuloma inguinale



Synonym(s)Lymphogranuloma inguinale; climaticbubo; Nicholas-Favre disease

DefinitionSexually transmitted chlamydial disease,characterized by genital ulceration andmarked regional lymphadenopathy

PathogenesisCaused by Chlamydia trachomatis, an obli-gate intracellular organism which travels


366 Lymphomatoid granulomatosis

through the lymphatics to multiply withinmacrophages in regional lymph nodes; riskfactors: anal intercourse, unprotected sex-ual intercourse; multiple sex partners, sexwith partners in endemic areas

Clinical manifestationPrimary stage: small, painless papule orherpetiform ulcer, usually on glans penis orvaginal wall, which heals within a few days;unilateral painful inguinal lymphadenopa-thy; horizontal group of inguinal nodesmost commonly involved; enlargement ofthe nodes above and below the inguinal lig-ament (groove sign)Tertiary stage: proctocolitis; perirectalabscess; fistulas; anal strictures; hyperpla-sia of intestinal and perirectal lymphatics;end result sometimes elephantiasis of thefemale genitalia, characterized by fibroticlabial thickening, or elephantiasis anddeformation of the penis in men

Differential diagnosisChancroid; syphilis; granuloma inguinale;cat-scratch disease; infectious mononucleo-sis; tuberculosis; tularemia; brucellosis;bubonic plague; lymphoma; metastasis;Crohn disease

TherapyDoxycycline; erythromycin, systemic

ReferencesMabey D, Peeling RW (2002) Lymphogranuloma

venereum. Sexually Transmitted Infections 78(2):90–92

Lymphomatoid granulomatosis

Synonym(s)Angiocentric lymphoproliferative lesion;polymorphic reticulosis

DefinitionSystemic angiodestructive lymphoprolifera-tive disease, characterized by prominentpulmonary involvement

PathogenesisProbably distinctive type of B-cell lym-phoma associated with exuberant, benign,T-cell reaction

Clinical manifestationSkin: patchy, occasionally painful, ery-thematous macules, papules, and plaquesinvolving gluteal regions and extremities;subcutaneous nodules which may ulceratePulmonary involvement: cough; dyspnea;hemoptysis; sputum production possiblyreflecting associated pneumoniaNeurological manifestations: lymphocyticinfiltration of the meninges, cerebral ves-sels, and peripheral nerves; mass lesions;mental status changes, ataxia, hemiparesis,seizures, distal sensory neuropathy, monon-euritis multiplex; associated with Sjögrensyndrome, chronic viral hepatitis, rheuma-toid arthritis, renal transplantation, andhuman immune deficiency virus (HIV)infectionLethal midline granuloma variant: destruc-tive lesions of midface, nasal cavity, nasalsinuses

Differential diagnosisBronchocentric granulomatosis; Churg-Strauss disease; sarcoidosis; Wegener’sgranulomatosis; non-Hodgkin’s lymphoma

TherapySystemic corticosteroids with or withoutchemotherapy

ReferencesJaffe ES, Wilson WH (1997) Lymphomatoid gran-

ulomatosis: pathogenesis, pathology and clini-cal implications. Cancer Surveys 30:233–248


Lymphomatoid papulosis 367

L

Lymphomatoid papulosis

Synonym(s)Macaulay disease; Macaulay's disease

DefinitionChronic lymphoproliferative disease of theskin, characterized by recurrent crops ofpapules that may ulcerate and heal withscarring

PathogenesisUnknown; CD30 (Ki-1) positive lymphopro-liferative disorder; possibly either benignchronic disorder of activated T cellsresponding to external or internal stimulior low-grade T-cell lymphoma localized toskin

Clinical manifestationCrops of mildly pruritic red papules evolv-ing into red-brown, often hemorrhagic, ves-

icles or pustules with necrotic crust; heal-ing with depressed scars; most commondistribution on trunk and extremities; asso-ciated systemic lymphoma in some patients

Differential diagnosisPityriasis lichenoides et varioliformis acuta;leukemia cutis; drug eruption; pityriasislichenoides et varioliformis acuta (Mucha-Habermann disease); cutaneous B-cell lym-phoma; Hodgkin’s disease; scabies; insectbite reaction; pseudolymphoma; Langer-hans cell histiocytosis; miliaria; folliculitis

TherapyMethotrexate�; photochemotherapy

ReferencesKarp DL, Horn TD (1994) Lymphomatoid papulo-

sis. Journal of the American Academy of Der-matology 30(3):379–395


M

Macaulay disease

�


Macaulay's disease

�


Macroglobulinemia

�

Waldenström macroglobulinemia

Macular atrophy of the skin

�


Madelung’s disease

�


Madura foot

�

Eumycetoma

�

Mycetoma

Maduromycosis

�

Eumycetoma

�

Mycetoma

Maffucci syndrome

Synonym(s)

Enchondromatosis

;

dyschondrodysplasiawith hemangiomas

;

enchondromatosis withmultiple cavernous hemangiomas

Definition

Disorder characterized by benign cartilagi-nous tumors (enchondromas), bonedeformities, and hemangiomas

Pathogenesis

Unknown


Hemangiomas in various areas of the body,including leptomeninges, eyes, pharynx,tongue, trachea, and intestines; enchondro-mas, usually on the hands


Kaposi’s sarcoma; Klippel-Trenaunay-Weber syndrome; dyschondrodysplasiawith hemangiomas; enchondromatosiswith multiple cavernous hemangiomas;


370 Majocchi granuloma

Gorham syndrome; Ollier disease; proteussyndrome

Therapy

None for asymptomatic lesions; surgicalrepair for bone fractures, as needed

References

Kuwahara RT, Skinner RB Jr (2002) Maffucci syn-drome: a case report. Cutis 69(1):21–22

Majocchi granuloma

Synonym(s)

Majocchi’s granuloma

;

granuloma tricho-phyticum

;

granuloma tricofitico

Definition

Nodular perifolliculitis secondary to a der-matophyte infection

Pathogenesis

Type IV hypersensitivity reaction, mostcommonly due to Trichophyton rubruminfection; possibly a response to the organ-ism itself or non-specific inflammatoryresponse to follicular contents


Develops on any hair-bearing area, butmost often on the scalp, face, forearms, dor-

sal aspect of hands, and legs; solitary ormultiple, well-circumscribed, oval, scalyplaques with perifollicular papules andpustules


Folliculitis; pseudofolliculitis barbae; sca-bies; acne keloidalis; psoriasis; lupus ery-thematosus; acquired perforating disease;contact dermatitis; coccidioidomycosis;rosacea; herpes simplex virus infection;ecthyma

Therapy

Terbinafine; itraconazole; azole antifungalagents

References

Elgart ML (1996) Tinea incognito: an update on Majocchi granuloma. Dermatologic Clinics 14(1):51–55

Majocchi’s disease

�

Majocchi granuloma

Majocchi’s granuloma

�

Majocchi granuloma

Mal de Meleda

Synonym(s)

Acroerythrokeratoderma

;

keratoderma pal-moplantaris transgradiens

Definition

Keratoderma of the palms and soles occur-ing as a genetic disease, mainly in residentsof the island of Meleda

Majocchi granuloma.

Red-brown plaque on the hand, studded with follicular papules and pustules


Male pattern baldness 371

M

Pathogenesis

Recessive or variable dominant mode oftransmission; exact genetic defect unknown


Keratoderma of the palms and soles, withextension onto the dorsa of the affectedlimbs; extension to the elbows and knees;associated eczema in many patients; hyper-hidrosis; nail thickening and koilonychia


Olmsted syndrome; keratosis lichenoideschronica striata; mutilating keratoderma(Vohwinkel’s syndrome); progressive pal-moplantar keratoderma; tylosis (Unna-Thost syndrome)

Therapy

Keratolytic therapy, such as 6% salicylicacid in 70% propylene glycol; acetretin

References


Mal de pinto

�

Pinta

Malacoplakia

�

Malakoplakia, cutaneous

Malakoplakia, cutaneous

Synonym(s)

Malacoplakia

Definition

Immunodeficiency disease characterized byvariable and non-specific skin lesions andassociated with macrophage dysfunction

Pathogenesis

Caused by inadequate bacterial killing bymacrophages or monocytes that exhibitdefective phagolysosomal activity; risk fac-tors: chronic corticosteroid use, rheuma-toid arthritis, diabetes mellitus, and organtransplantation


Yellow-to-pink papules, nodules, or ulcers,most commonly in perianal or inguinalareas, the buttocks and abdominal wall, orin mucous membranes; occasional second-ary infection, most commonly Escherichiacoli; chronic, benign, self-limited course


Squamous cell carcinoma; sarcoidosis;Langerhans cell histiocytosis; lymphomahistiocytoma; granular cell tumor; furun-cle; actinomycosis; botryomycosis

Therapy

Surgical excision

�

; ciprofloxacin; trimetho-prim-sulfamethoxazole: 160 mg TMP/800 mg SMZ PO twice daily for 7–14 days

References

Remond B, Dompmartin A, Moreau A, Esnault P, Thomas A, Mandard JC, Leroy D (1994) Cuta-neous malacoplakia. International Journal of Dermatology 33(8):538–542

Male genital lichen sclerosus

�


Male pattern baldness

�



372 Male Turner syndrome

Male Turner syndrome

�

Noonan’s syndrome

Malherbe, calcifying epithelioma of

�

Pilomatricoma

Mali’s disease

�

Acroangiodermatitis

Malignancy of fat cells

�

Liposarcoma

Malignant angioendotheliomatosis

�


Malignant angioma

�

Angiosarcoma


Synonym(s)

Degos’ disease

;

Degos’ syndrome

;

Kohl-meier-Degos syndrome

;

papulosis atrophi-

cans maligna

;

macular atrophy of the skin;fatal cutaneointestinal syndrome

;

lethalcutaneous and gastrointestinal arterialthrombosis

Definition

Multisystem disorder involving small-cal-iber blood vessels, characterized by narrow-ing and occlusion of the lumen by intimalproliferation and thrombosis, leading toischemia and infarction in the involvedorgans

Pathogenesis

Unknown


Skin findings: multiple, small, asympto-matic papules appearing in crops and pri-marily involving the trunk and limbs whilesparing the palms, soles, face, and scalp;pinkish papules become umbilicated, withdepressed centers, and turn porcelain-whiteGastrointestinal manifestations: nonspe-cific; including abdominal pain, abdominaldistention, nausea, vomiting, diarrhea orconstipation, weakness, fatigue, weight loss,or symptoms of malabsorption; in latestage, gastrointestinal hemorrhage, bowelinfarction, and perforationNeurological findings: involvement of bothcentral and peripheral nervous systems;paresthesias of the face and extremities,headaches, dizziness, seizures, hemiplegia,aphasia, paraplegia, and gaze palsy


Pyoderma gangrenosum; lupus erythema-tosus; Crohn disease; polyarteritis nodosa;thromboangiitis obliterans; lichen planus;morphea; lichen sclerosus; burn or otherskin trauma

Therapy

No effective therapy, including anticoagu-lants, antiplatelet drugs such as aspirin anddipyridamole, corticosteroids, immunosup-pressants, sulfonamide, tetracycline, andpenicillin


Malignant nonchromaffin paraganglioma 373

M

References

Demitsu T, Nakajima K, Okuyama R, Tadaki T (1992) Malignant atrophic papulosis (Degos' syndrome). International Journal of Dermatol-ogy 31(2):99–102

Malignant carcinoid syndrome

Synonym(s)

Carcinoid syndrome

Definition

Constellation of symptoms seen in patientswith metastases from carcinoid tumors

Pathogenesis

Primary tumors arise from neuroendo-crine cells secreting serotonin; variety ofvasoactive peptides produced, causing clin-ical symptoms


Flushing of the face and neck, sometimesbrief (e.g., 2–5 min) or lasting for severalhours; fixed telangiectasia and/or viola-ceous hue, primarily on the face and neck,most marked in the malar area; tachycardiawithout significant change in blood pres-sure


Urticaria; anaphylaxis; angioedema; pheo-chromocytoma; mastocytosis; pellagra

Therapy

Antihistamines, first generation; octreotide100 mcg subcutaneously 3–4 times per day;various cancer chemotherapy regimens

References

Bax ND, Woods HF, Batchelor A, Jennings M (1996) Clinical manifestations of carcinoid dis-ease. World Journal of Surgery 20(2):142–146

Malignant down

�

Hypertrichosis lanuginosa

Malignant endothelioma

�

Angiosarcoma

Malignant endovascular papillary angioendothelioma

�


Malignant melanoma

�

Melanoma

Malignant mole

�

Melanoma

Malignant nonchromaffin paraganglioma

�



374 Malignant organoid granular cell myoblastoma

Malignant organoid granular cell myoblastoma

�


Malignant papillary dermatosis

�

Paget’s disease

Malignant pustule

�

Anthrax, cutaneous

Malignant rhabdomyoma

�

Rhabdomyosarcoma

Malignant tumors with eccrine differentiation

�

Eccrine carcinoma

Malleus

�


Mallorca acne

�

Acne aestivalis

Marasmus

Synonym(s)

Protein energy malnutrition

Definition

Type of malnutrition resulting from chronicinadequate consumption of protein andenergy, characterized by wasting of muscle,fat, and other body tissue

Pathogenesis

Negative energy balance from decreasedenergy intake, increased energy expendi-ture, or both


Occurs mostly in young children; shrunkenwasted appearance, with withdrawn behav-ior; loss of muscle and subcutaneous fatmass


Kwashiorkor; underlying systemic malig-nancy

TherapyNutritional supplementation�

ReferencesAkner G, Cederholm T (2001) Treatment of pro-

tein-energy malnutrition in chronic nonmalig-nant disorders. American Journal of Clinical Nutrition 74(1):6–24

Marchiafava-Micheli syndrome

� Paroxysmal nocturnal hemoglobinuria

Marfan disease

� Marfan syndrome


Massage alopecia 375

M

Marfan syndrome

Synonym(s)Marfan's syndrome, Marfan disease,Marfan's disease

DefinitionInherited connective tissue disorder char-acterized by abnormalities in skeletal sys-tem, cardiovascular system, eyes, and skin

PathogenesisAutsomal dominant trait; mutations in thefibrillin-1 (FBN1) gene located on chromo-some 15q21.1; production of abnormal fibril-lin-1 monomers from the mutated gene dis-rupt multimerization of fibrillin-1 and pre-vent microfibril formation, leading toabnormal connective tissue structure

Clinical manifestationSkin findings: striae; hyperextensible skin;elastosis perforans serpiginosa; high,arched palate; poor wound healingSkeletal findings: joint hypermobility; pec-tus excavatum; scoliosis; long arms and legsOcular findings: ectopic lens; early cataractsCardiopulmonary findings: aortic root dila-tation and dissection; aortic valve prolapse;spontaneous pneumothoraxNeurologic findings: dura ectasia

Differential diagnosisEhlers-Danlos syndrome; Klinefelter’s syn-drome; fragile X syndrome

TherapyNone for skin changes

ReferencesAburawi EH, O'Sullivan J, Hasan A (2001) Mar-

fan's syndrome: a review. Hospital Medicine (London) 62(3):153–157

Marfan's disease

� Marfan syndrome

Marfan's syndrome

� Marfan syndrome

Marjolin ulcer

Synonym(s)Marjolin’s ulcer

DefinitionMalignant tumor that arises in chronicburn wounds or other skin ulcerations

ReferencesPhillips TJ, Salman SM, Bhawan J, Rogers GS

(1998) Burn scar carcinoma. Diagnosis and management. Dermatologic Surgery 24(5):561–565

Marjolin’s ulcer

� Marjolin ulcer

Market men’s disease

� Tularemia

Mask of pregnancy

� Melasma

Massage alopecia

� Traction alopecia


376 Mastocytosis

Mastocytosis

Synonym(s)Urticaria pigmentosa; mastocytosis syn-drome

DefinitionDisorder characterized by mast cell prolif-eration and accumulation within variousorgans, including the skin

PathogenesisMay be abnormal mast cell response tounknown stimuli; increased local concen-trations of mast cell growth factors stimu-late mast cell proliferation; systemic mani-festations reflect the release of mast cell-derived mediators, such as histamine, pros-taglandins, heparin, neutral proteases, andacid hydrolases

Clinical manifestationMost common in children, who have 25–100 red-brown macules or barely elevatedpapules, usually over the trunk; lesionbecomes a wheal when rubbed (Darier’ssign); solitary mastocytoma: usuallyappears within first month of life; rubbery,yellow to brown, plaques, urticate with orwithout vesiculation after rubbing (bullousurticaria pigmentosa); telangiectasia macu-laris eruptiva perstans: brown macules andtelangiectasias with erythema, often overupper trunk; associated with peptic ulcerdisease; diffuse mastocytosis: bullae ininfancy, replaced by doughy skin, with gen-eralized pruritus; dermatographism, bullaeafter minor skin trauma; mast cell infiltra-tion of liver, spleen, skeleton, and gastroin-testinal tract; flushing syndrome, mostcommon in early life

Differential diagnosisSpitz nevus; juvenile xanthogranuloma;amyloidosis; sarcoidosis; granuloma annu-lare; melanocytic nevus; fixed drug erup-tion; insect bite reaction; lymphoma; Jess-ner lymphocytic infiltrate; lentigo; ber-

loque dermatitis; Langerhans cellhistiocytosis

TherapyAntihistamines, second generation�; pho-tochemotherapy; corticosteroids, topical,super potency

ReferencesHartmann K, Bruns SB, Henz BM (2001) Masto-

cytosis: review of clinical and experimental as-pects. Journal of Investigative Dermatology Symposium Proceedings 6(2):143–147

Mastocytosis syndrome

� Mastocytosis

McCune-Albright Syndrome

Synonym(s)Albright syndrome; Albright’s syndrome;osteitis fibrosa disseminata; fibrous dyspla-sia of bone; polyostotic dysplasia;polyostotic fibrous dysplasia; osteitis fib-rosa cystica; Fuller-Albright syndrome;Albright-Sternberg-McCune syndrome;brown spot syndrome

DefinitionFibrous dysplasia of bone; sexual precocity;hyperpigmentation

PathogenesisMutation in the GNAS1 gene coding forguanine nucleotide-binding protein G alphasubunit (protein Gs); mosaic pattern ofautonomously functioning clones of cells inthe affected organs

Clinical manifestationHyperpigmented patches, often followingBlaschko’s lines; precocious puberty; otherendocrine dysfunction: thyroid storm (par-ticularly during general anesthesia), tach-yarrhythmia, and fever; cushingoid habi-


Median rhomboid glossitis 377

M

tus; acromegaly; hirsutism; galactorrhea;skeletal deformities

Differential diagnosisHyperpigmented skin lesions: neurofi-bromatosis; tuberous sclerosis; Bloom syn-drome; ataxia-telangiectasia; Russell-Silversyndrome; Fanconi anemia; precociouspuberty; ovarian/testicular tumors; adrenaltumors; congenital adrenal hyperplasia;exogenous estrogens/androgen intake

TherapySurgical excision of hyperfunctional endo-crine tissue if severe endocrine imbalancepresent

Referencesde Sanctis C, Lala R, Matarazzo P, Balsamo A, et

al. (1999) McCune-Albright syndrome: A longi-tudinal clinical study of 32 patients. Journal of Pediatric Endocrinology 12(6):817–826

MD Forte facial cream


Measles

� Rubella� Rubeola

Median canal dystrophy

� Median nail dystrophy

Median nail dystrophy

Synonym(s)Median canal dystrophy

DefinitionAcquired nail plate disorder characterizedby longitudinal split in the center of the nailplate

PathogenesisSometimes related to trauma of the proxi-mal nail fold area from habitual picking,etc.

Clinical manifestationLongitudinal split appears in center of nailplate; several fine cracks project from theline laterally, giving the appearance of firtree; thumb most often affected; spontane-ous remission after months to years, withrecurrences possible

Differential diagnosisUnderlying anatomic defects, includingmucous cyst, squamous cell carcinoma;melanoma; wart; exostosis; onychomycosis;psoriasis; lichen planus

TherapyNone

ReferencesGriego RD, Orengo IF, Scher RK (1995) Median

nail dystrophy and habit tic deformity: are they different forms of the same disorder? Interna-tional Journal of Dermatology 34(11):799–800

Median rhomboid glossitis

Synonym(s)Central papillary atrophy; posterior lingualpapillary atrophy

DefinitionDefective embryonic posterior dorsaltongue point of fusion, leaving a rhomboid-shaped, smooth, erythematous mucosalacking in papillae or taste buds

PathogenesisOnset occurring during embryonic tonguedevelopment


378 Mediterranean spotted fever

Clinical manifestationSmooth, flat, or slightly lobulated plaque onposterior midline of the dorsum of thetongue, just anterior to the V-shaped group-ing of the circumvalate papillae; secondarychronic candida infection

Differential diagnosisSquamous cell carcinoma; black hairytongue; lingual thyroid; tertiary syphilis;tuberculosis; granular cell tumor

TherapyAzole antifungal troches for candida super-infection

ReferencesCarter LC (1990) Median rhomboid glossitis: re-

view of a puzzling entity. Compendium 11(7):446, 448–451

Mediterranean spotted fever


Meibomian cyst

� Chalazion

Melandodermic leukodystrophy


Melanoacanthoma

Synonym(s)Benign mixed tumor of melanocytes andmalpighian cells; melanoepithelioma;melanoacanthosis

DefinitionBenign mixed tumor of keratinocytes andmelanocytes

PathogenesisTrauma a possible factor in this reactiveprocess; may be a seborrheic keratosis vari-ant

Clinical manifestationSolitary, hyperpigmented or verrucous,round or oval papule, plaque, cutaneoushorn, or nodule, usually on trunk, lip, oreyelid; also occur in oral mucosa

Differential diagnosisMelanocytic nevus; melanoma; seborrheickeratosis; wart; actinic keratosis; pig-mented basal cell carcinoma; mucosal mela-nosis

TherapyCryotherapy; destruction by electrodessica-tion and curettage; simple excision

ReferencesTomich CE, Zunt SL (1990) Melanoacanthosis

(melanoacanthoma) of the oral mucosa. Jour-nal of Dermatologic Surgery & Oncology 16(3):231–236

Melanoacanthosis

� Melanoacanthoma

Melanoepithelioma

� Melanoacanthoma

Melanoid mycetoma

� Eumycetoma


Melanosis faciei feminae 379

M

Melanoma

Synonym(s)Malignant melanoma; malignant mole

DefinitionMalignant tumor of melanocytes

PathogenesisMultiple contributing factors: (1) fair com-plexion; (2) excessive childhood sun expo-sure and blistering childhood sunburns;(3) increased number of common acquiredand atypical moles; (4) family history ofmelanoma; (5) xeroderma pigmentosum;(6) familial atypical mole melanoma syn-drome

Clinical manifestationOccurs most commonly on the trunk inwhite males and the lower legs and back inwhite females, in pigmented races; mostcommon sites are plantar foot, subungual,palmar, and mucosal sitesSuperficial spreading subtype: most com-mon, occurring in approximately 70% ofpatients; a flat or slightly elevated papule orplaque, with variegate pigmentation (black,brown, blue, or pink discoloration), usuallygreater than 6 mm in diameter; irregularasymmetric bordersNodular subtype: most commonly seen onthe legs and trunk; rapid growth of a dark

brown-to-black papule or dome-shapednodule, which may be friable and ulcerate;lentigo maligna melanoma: arises inintraepithelial precursor lesion, lentigomaligna; slow growing, irregular, pig-mented patch, located on the sun-damagedskin of head, neck, and arms of fair-skinnedolder individuals; over time, dark brown-to-black macular pigmentation or raisedblue-black nodules evolvesAcral lentiginous subtype: least commonvariety, but most common type in dark-skinned individuals; occurs on the palms,soles, or beneath the nail plate; subunguallesion presents as diffuse nail discolorationor longitudinal pigmented band within thenail plate, with pigment spreading to theproximal or lateral nail folds (Hutchinsonsign)

Differential diagnosisMelanocytic nevus, including atypicalmole; lentigo; seborrheic keratosis; pyo-genic granuloma; basal cell carcinoma;squamous cell carcinoma; dermatofibroma;cherry hemangioma; metastasis; keratoa-canthoma; chronic paronychia; subungualhematoma; melanonychia striata

TherapyWide local excision�; sentinel node biopsyand node dissection as needed for 1-4 mmdeep primary tumors; adjuvant interferon(IFN) alfa-2b for high risk primary tumorsor regional micrometastatic disease

ReferencesLang PG (2002) Current concepts in the manage-

ment of patients with melanoma. American Journal of Clinical Dermatology 3(6):401–426

Melanosis faciei feminae


Melanoma. Irregular pigmented plaque, with shades of red, white, and blue


380 Melanosis lenticularis progressiva

Melanosis lenticularis progressiva


Melanotic hyperpigmentation

� Postinflammatory hyperpigmenta-tion

Melasma

Synonym(s)Chloasma; mask of pregnancy; pregnancymask

DefinitionAcquired, chronic hypermelanosis, charac-terized by macular hyperpigmentation,mainly in sun-exposed skin

PathogenesisMultiple contributing factors, includinggenetic predispsition, sun exposure, hor-

monal stimulation; racial predisposition(e.g., common in Hispanics)

Clinical manifestationSymmetric, tan-brown, macular hyperpig-mentation; occurs in 1 of 3 patterns: centralfacial, malar, or mandibular; irregular,sharply marginated borders

Differential diagnosisBerloque dermatitis; lentigo; nevocellularnevus; polymorphous light reaction; lupuserythematosus; poikiloderma of Civatte;mastocytosis; Addison disease; lichen pla-nus; morphea; Riehl’s melanosis; postin-flammatory hyperpigmentation; drug-induced hyperpigmentation

TherapyHydroquinone; azelaic acid; tretinoin; sun-screen protection; chemical peel; laser ther-apy

ReferencesPandya AG, Guevara IL (2000) Disorders of hy-

perpigmentation. Dermatologic Clinics. 18(1):91–98

Melioidosis

� Glanders and melioidosis

Melkersson-Rosenthal syndrome


MEN IIB syndrome

� Mucosal neuroma syndrome

Melasma. Reticulated, brown plaque on the face


Menke’s kinky hair syndrome 381

M

MEN III syndrome


Meningococcal sepsis

� Meningococcemia

Meningococcemia

Synonym(s)Meningococcal sepsis

DefinitionAcute infection of the bloodstream andsubsequent vasculitis with the bacteriaNeisseria meningitidis

PathogenesisCaused by Neisseria meningitidis, an obli-gate, non-motile, aerobic, encapsulatedgram-negative diplococcus; host factors:deficiencies of terminal complement com-ponents C5 through C9 or properdin,immunoglobulin deficiency, asplenia, andHIV infection; vascular injury the result ofdirect toxic effects of organism and immu-nologic reaction

Clinical manifestationMay follow upper respiratory infection;associated with headache, nausea, vomit-ing, myalgias, and arthralgias; fulminantdisease: hemorrhagic eruption, hypoten-sion, and cardiac depression occur withinhours of initial presentation; skin findingsof petechiae on the extremities and trunk,sometimes generalizing; progression: pus-tules, bullae, and hemorrhagic plaques withcentral necrosis and stellate purpura with acentral “gun-metal” gray hue; neurologic

findings: headache; altered mental status;neck stiffness; irritability; seizures; nervepalsies; gait disturbance; gastrointestinalfindings: nausea and vomiting

Differential diagnosisBacterial sepsis other than that caused byNeisseria meningitidis, such as gonococ-cemia, Haemophilus influenzae, and Strep-tococcus pneumoniae; Rocky Mountainspotted fever; viral illnesses, especiallyenteroviruses; toxic shock syndrome; lept-ospirosis; hypersensitivity vasculitis; Hen-och-Schönlein purpura; polyarteritisnodosa; dermatomyositis; lupus erythema-tosus; coagulopathies; idiopathic purpurafulminans

TherapyPenicillin G in sensitive strains�: 300,000 Uper kg per day, up to 24 million U per dayIV in 4–6 divided doses until 5–7 days aftertemperature has returned to normal; ceftri-axone: 2 g IV or IM every 12 hours until 5–7days after temperature has returned to nor-mal; cefotaxime for adults: 1–2 g IV or IMevery 6–12 hours; for children <50 kg:50 mg/kg IV or IM every 8 hours

ReferencesLevine N, Kunkel M, Nguyen T, Ackerman L

(2002) Emergency Department Dermatology. Current Problems in Dermatology 14(6):183–220

Menke’s kinky hair syndrome

Synonym(s)Menkes kinky hair disease; kinky hair syn-drome; steely hair syndrome; tricho-poliodystrophy

DefinitionMultisystem disorder of copper metabo-lism, characterized by fine silvery wiry hair,


382 Merkel cell carcinoma

doughy skin, connective tissue abnormali-ties, and progressive neurologic deteriora-tion

PathogenesisX-linked recessive trait with gene locus inband Xq13.3; defect in intestinal coppertransport with associated low serum cop-per and ceruloplasmin levels, resulting in adeficiency in copper-dependent enzymeactivity; copper-dependent metalloen-zymes relevant to the clinical phenotype:tyrosinase (pigmentation of skin and hair),lysyl oxidase (elastin and collagen cross-linking), ascorbate oxidase (skeletal devel-opment), monoamine oxidase (possiblyresponsible for pili torti), superoxide dis-mutase (free-radical detoxification),dopamine beta-hydroxylase (catecho-lamine production), peptidyl-glycine alpha-amidating mono-oxygenase (bioactivationof peptide hormones), and cytochrome coxidase (electron transport and possiblyresponsible for hypothermia)

Clinical manifestationSkin – hypopigmented, pale, cutis mar-morata; lax doughy skin of cheeks; cupid'sbow upper lipHair – pili torti; trichorrhexis nodosa; light-colored, sparse, short, brittle, kinky, steelwool-like hair; sparse, broken eyebrows andeyelashesAbnormal facies; musculoskeletal abnor-malities; progressive neurologic deteriora-tion

Differential diagnosisEctodermal dysplasia; argininosuccinic aci-duria; Björnstad syndrome; Crandall syn-drome; Salti-Salem syndrome; Tay syn-drome; Conradi-Hünermann chondrodys-plasia punctata; Bazex syndrome;citrullinemia; hypohidrotic ectodermal dys-plasia; Salamon syndrome; tricho-odon-tonychial dysplasia with pili torti; pili tortiand enamel hypoplasia

TherapyNone for skin and hair problems

ReferencesKodama H, Murata Y, Kobayashi M (1999) Clinical

manifestations and treatment of Menkes dis-ease and its variants. Pediatrics International 41(4):423–429

Merkel cell carcinoma

Synonym(s)Trabecular carcinoma; small cell carci-noma of the skin; primary cutaneous neu-roendocrine carcinoma

DefinitionAggressive primary cutaneous neoplasmwith neuroendocrine differentiation

PathogenesisCell of origin may be epidermal MerkelCell, a dermal Merkel Cell equivalent, aneural-crest-derived cell of the APUD(amine precursor uptake and decarboxyla-tion) system, or a residual epidermal stemcell; chronic exposure to solar ultravioletradiation possibly a co-factor

Clinical manifestationSingle, painless, firm, shiny, red or viola-ceous papule, with predilection for individ-uals with fair skin; most common in sev-enth decade and older; occurs most com-monly in the head and neck region andextremities; regional nodal metastases firstsite of dissemination; increased incidencein immunocompromised patients

Differential diagnosisSquamous cell carcinoma; basal cell carci-noma; melanoma; metastasis; Kaposi’s sar-coma; hemangioma; dermatofibroma; lym-phoma

TherapyWide local excision�; Mohs micrographicsurgery; regional lymph node dissection;radiation therapy for local palliation


Methoxsalen 383

M

ReferencesGoessling W, McKee PH, Mayer RJ (2002) Merkel

cell carcinoma. Journal of Clinical Oncology 20(2):588–598

Methotrexate

Trade name(s)Rheumatrex


Drug classAnti-metabolite

Mechanism of actionImmunosuppressive: inhibits dihydrofolatereductase; inhibits lymphocyte prolifera-tion

Dosage form2.5 mg tablet; 25 mg per ml solution forintramuscular injection


Common side effectsCutaneous: stomatitis, photosensitivity,skin eruption, alopeciaGastrointestinal: nausea, vomitingLaboratory: elevated liver functionenzymes

Serious side effectsBone marrow: marrow suppressionCutaneous: Stevens-Johnson syndrome,toxic epidermal necrolysis, exfoliative der-matitis, radiation recall reactionsPulmonary: pulmonary fibrosis

Drug interactionsAcitretin; COX-2 inhibitors; salicylates;non-steroidal anti-inflammatory agents;penicillins; sulfonamides; trimethoprim

Contraindications/precautionsHypersensitivity to drug class or compo-nent; pregnancy; alcohol abuse; severe liverdysfunction; immunodeficiency syn-dromes; caution in patients with impairedrenal function or ulcerative colitis


drugs. Dermatologic Clinics 19(1):105–118

Methoxsalen

Trade name(s)Oxsoralen Ultra; Oxsoralen lotion

Generic availableNo

Drug classPsoralen

Mechanism of actionSuppression of DNA synthesis; photo-immunologic effects; selective cytotoxicity;melanocyte stimulation

Dosage form10 mg capsules; 1% solution for dilution


Common side effectsCutaneous: phototoxic reaction, exanthem,herpes simplex virus infection recurrence,photo-aging after chronic useGastrointestinal: nausea, vomiting, hepatictoxicity

Serious side effectsCutaneous: carcinogenesisOcular: cataract formation

Drug interactionsDoxycycline; fluoroquinolones; carbamaze-pine; phenytoin


384 Methoxsalen

Methotrexate. Dermatologic indications and dosage


Bullous pemphigoid 7.5–25 mg PO as a single dose weekly

5–15 mg PO as a single weekly dose

Chronic graft versus host disease

7.5–25 mg PO as a single dose weekly


Dermatomyositis 7.5–25 mg PO as a single dose weekly





Lupus erythematosus 7.5–25 mg PO as a single dose weekly



5–7.5 mg PO as a single weekly dose

2.5–5 mg PO as a single weekly dose

Mycosis fungoides 7.5–25 mg PO as a single dose weekly


Pemphigus vulgaris 7.5–25 mg PO as a single dose weekly


Pityriasis lichenoides 5–7.5 mg PO as a single weekly dose

2.5–5 mg PO as a single weekly dose

Pityriasis rubra pilaris 7.5–25 mg PO as a single dose weekly


Progressive systemic sclerosis



Psoriasis 7.5–25 mg PO as a single dose weekly





Reiter syndrome 7.5–25 mg PO as a single dose weekly





Sarcoidosis 7.5–25 mg PO as a single dose weekly


Sézary syndrome 7.5–25 mg PO as a single dose weekly


Vasculitis, including polyarteritis nodosa




Metronidazole, topical 385

M


ReferencesLaube S, George SA (2001) Adverse effects with

PUVA and UVB phototherapy. Journal of Der-matological Treatment 12(2):101–105

Lim HW, Edelson RL (1995) Photopheresis for the treatment of cutaneous T-cell lymphoma. He-matology – Oncology Clinics of North America 9(5):1117–1126

Metronidazole, topical

Trade name(s)MetroGel; MetroCream; MetroLotion; Nori-tate


Drug classNitroimidazole antibiotic

Mechanism of actionDNA disruption and inhibition of nucleicacid synthesis (may not be mechanism inskin disease treatment)

Dosage form0.75% cream, gel; 1% cream


Common side effectsCutaneous: burning sensation, erythema,skin eruption



Methoxsalen. Dermatologic indications and dosage

Disease Adult dose Child dose

Component of photochemotherapy – psoriasis; Reiter syndrome; cutaneous T cell lymphoma (mycosis fungoides; Sézary syndrome; vitiligo; polymorphous light eruption; solar urticaria; chronic actinic dermatitis; morphea; linear scleroderma; graft versus host disease; lymphomatoid papulosis

Systemic photochemotherapy – 0.4–0.6 mg per kg PO 1.5 hours before exposure to ultraviolet A light, either via light box, outdoor sunlight, or photopheresis; topical therapy – 0.1% lotion applied 30 minutes before exposure to ultraviolet A light

Systemic photochemotherapy – 0.4–0.6 mg per kg PO 1.5 hours before exposure to ultraviolet A light, either via light box, outdoor sunlight, or photopheresis; topical therapy – 0.1% lotion applied 30 minutes before exposure to ultraviolet A light

Component of photopheresis – T-cell lymphoma (mycosis fungoides; Sézary syndrome)

0.4–0.6 mg per kg PO 1.5 hours before exposure to ultraviolet A light

0.4–0.6 mg per kg PO 1.5 hours before exposure to ultraviolet A light


386 Michelin tire baby syndrome


ReferencesCohen AF, Tiemstra JD (2002) Diagnosis and

treatment of rosacea. Journal of the American Board of Family Practice 15(3):214–217

Michelin tire baby syndrome

Synonym(s)Michelin tire syndrome; Kunze Riehm syn-drome

DefinitionHeterogeneous group of disorders charac-terized by ringed creases of the extremities

PathogenesisAutosomal dominant trait; at least two dis-tinct chromosomal abnormalities

Clinical manifestationDeep, gyrus-like skin folds on the back; cir-cumferential, deep skin folds of limbs, withspontaneous resolution of skin creases inchildhood; loose, thick skin; xanthomasand/or lipomas; hypertrichosis with under-lying smooth muscle hamartoma; cleft pal-ate; neuroblastoma; congenital heart defects

Differential diagnosisNevus lipomatosis

TherapyNone

ReferencesGlover MT, Malone M, Atherton DJ (1989) Miche-

lin-tire baby syndrome resulting from diffuse

smooth muscle hamartoma. Pediatric Derma-tology 6(4):329–331

Michelin tire syndrome

� Michelin tire baby syndrome

Miescher syndrome 2


Miescher-Melkersson-Rosenthal syndrome


Miescher’s cheilitis granulomatosa


Miescher’s granulomatosis

� Actinic granuloma� Cheilitis granulomatosa

Metronidazole, topical. Dermatologic indications and dosage


Rosacea Apply once daily Apply once daily


Miliaria 387

M

Migratory necrolytic erythema

DefinitionMigratory eruption on face, abdomen, peri-neum, buttocks, or lower extremities, usu-ally associated with underlying glucagon-oma

� Glucogonoma

ReferencesSchwartz RA (1997) Glucagonoma and pseudog-

lucagonoma syndromes. International Journal of Dermatology 36(2):81–89

Mikulicz disease

� Rhinoscleroma

Miliaria

Synonym(s)Prickly heat; sudamina; heat rash; lichentropicus; tropical anhidrosis

DefinitionDisorder of the eccrine sweat glands oftenoccurring in conditions of increased heatand humidity, caused by blockage of thesweat ducts that results in the leakage ofeccrine sweat into skin

PathogenesisOcclusion of the skin, due to clothing orbandages, resulting in pooling of sweat onthe skin surface and overhydration of thestratum corneum; in susceptible persons,including infants, with relatively immatureeccrine glands, stratum corneum overhy-dration causes transient blockage of theacrosyringium, resulting in leakage ofsweat; other contributing factors: immatu-

rity of the sweat ducts in neonates, lack ofacclimitization, occlusive clothing, hot andhumid conditions, vigorous exerciose, andbacterial overgrowth

Clinical manifestationMiliaria crystallina: usually affects neonatesand adults who are febrile or who recentlymoved to a tropical climate; asymptomatic,clear, superficial vesicles appear in crops,often confluent, and without surroundingerythema; rupture easily and resolve withsuperficial, branny desquamation; occurwithin days to weeks of exposure to hotweather and disappear within hours todays; in infants, lesions occur on the head,neck, and upper part of the trunk; in adults,lesions appear on the trunkMiliaria rubra: occurs in hot, humid envi-ronments; pruritic or painful, small, dis-crete, non-follicular, erythematous papulesand vesicles; lesions on the neck and in thegroin and axillae; lesions on covered skinsubject to friction, such as the neck, scalp,upper part of the trunk, and flexures inadultsMiliaria profunda: occurs in those in a trop-ical climate who have had repeated epi-sodes of miliaria rubra; asymptomatic,firm, flesh-colored papules, usually on thetrunk, developing within minutes or hoursafter the stimulation of sweating andresolves quickly after removal of stimulusthat caused sweating; increased sweating inunaffected skin; lymphadenopathy; hyper-pyrexia and symptoms of heat exhaustion,including dizziness, nausea, dyspnea, andpalpitations

Differential diagnosisFolliculitis; milia; viral exanthem; cutane-ous candidiasis; erythema toxicum; insectbite reaction; scabies; foreign body reac-tion; drug eruption; cholinergic urticaria

TherapyMiliaria crystallina: no therapy indicatedMiliaria rubra: removal of occlusive cloth-ing; limiting of activity; air conditioningMiliaria profunda: removal of occlusiveclothing; limited activity; air conditioning;


388 Miliaria cystallina

anhydrous lanolin lotion applied 2–3 timesdaily and before activity that may produceexcess sweating

ReferencesWenzel FG, Horn TD (1998) Nonneoplastic disor-

ders of the eccrine glands. Journal of the Amer-ican Academy of Dermatology 38(1):1–17

Miliaria cystallina

� Miliaria

Miliaria profunda

� Miliaria

Miliaria pustulosa

� Miliaria

Miliary tuberculosis of the skin


Milium

Synonym(s)None

DefinitionSmall, benign, keratin-filled cyst

PathogenesisDerived from the pilosebaceous follicle; pri-mary lesions arise from vellus hair follicles;

secondary milia result from damage topilosebaceous unit after skin trauma

Clinical manifestationUniform, pearly-white to yellowish, small,domed papules, often in groups; primarymilia: usually on the face of newborns; seenaround the eye in children and adults; sec-ondary lesions: arise after blistering ortrauma, including bullous pemphigoid,inherited and acquired epidermolysis bul-losa, bullous lichen planus, porphyria cuta-nea tarda, and burns

Differential diagnosisAcne vulgaris; flat wart; syringoma; trich-oepithelioma; xanthoma

TherapyIncision and drainage; light hyfrecation


diseases. Part I. Journal of the American Acad-emy of Dermatology. 39(2 Pt 1):149–171

Minocycline

Trade name(s)Minocin; Dynacin; Vectrin


Drug classTetracycline

Mechanism of actionAntibiotic activity: protein synthesis inhibi-tion by binding to the 30S ribosomal subu-nit; anti-inflammatory activity: unclearmechanism

Dosage form50 mg, 75 mg, 100 mg tablets



Minocycline 389

M

Common side effectsCutaneous: photosensitivity, stomatitis,oral candidiasis, urticaria or other vascularreactionGastrointestinal: nausea and vomiting,diarrhea, esophagitisNeurologic: tinnitus, dizziness, drowsiness,headache, ataxia

Serious side effectsGastrointestinal: pseudomembranous coli-tis, hepatotoxicity

Hematologic: neutropenia, thrombocytope-niaNeurologic: pseudotumor cerebri


Contraindications/precautionsHypersensitivity to drug class or compo-nent; pregnancy; patient < 8 years old; cau-

Minocycline. Dermatologic indications and dosage


Acne vulgaris 50–100 mg PO twice daily > 8 years old – 50–100 mg PO twice daily


50–100 mg PO twice daily > 8 years old – 50–100 mg PO twice daily

Bullous pemphigoid 50–100 mg PO twice daily > 8 years old – 50–100 mg PO twice daily

Confluent and reticulate papillomatosis of Gougerot and Carteaud




Folliculitis 50–100 mg PO twice daily > 8 years old – 50–100 mg PO twice daily




100 mg PO twice daily for 4–6 weeks after clinical resolution

> 8 years old – 50–100 mg PO twice daily for 4–6 weeks after clincial resolution

Nocardiosis 100-200 mg PO daily for 2–4 weeks > 8 years old – 100-200 mg PO daily for 2–4 weeks

Pemphigus foliaceus 50–100 mg PO twice daily > 8 years old – 50–100 mg PO twice daily

Perioral dermatitis 50–100 mg PO twice daily for at least 30 days


Rosacea 50–100 mg PO twice daily for at least 30 days


Rosacea 50–100 mg PO twice daily > 8 years old – 50–100 mg PO twice daily


390 Minoxidil, topical

tion in patients with impaired renal or liverfunction



Minoxidil, topical

Trade name(s)Rogaine


Drug classPeripheral vasodilator

Mechanism of actionUnclear; may involve vasodilatation and/oranti-androgen mechanisms

Dosage form2%, 5% solution


Common side effectsCutaneous: irritant dermatitis, hypertricho-sis



Contraindications/precautionsHypersensitivity to drug class or compo-nent; caution in patients over 50 years old

ReferencesPrice VH (1999) Treatment of hair loss. New Eng-

land Journal of Medicine 341(13):964–973


Synonym(s)Sharp syndrome; Sharp’s syndrome

DefinitionDisorder characterized by elements of sev-eral connective tissue diseases, such as: sys-temic lupus erythematosus, systemic scle-rosis, dermatomyositis, polymyositis, andSjögren syndrome

PathogenesisProbable autoimmune phenomenon withantibodies against the U1-RNP complex ingenetically predisposed individuals

Clinical manifestationSkin findings: Raynaud phenomenon; sau-sage-shaped fingers; swelling of the dorsa ofthe hands; abnormal capillaries in the nailfold; with palpable red papules or plaquessimilar to chronic cutaneous lupus ery-thematosus; alopecia; facial erythema; per-iungual telangiectasiaMusculoskeletal: arthralgia and arthritis;myalgia; myositis; muscle weakness

Minoxidil, topical. Dermatologic indications and dosage



Apply twice daily Not indicated

Anagen effluvium Apply twice daily Not indicated


Molluscum contagiosum 391

M

Gastrointestinal: dysphagia and dysfunc-tion of esophageal motilityPulmonary: pleural effusion; interstitialpulmonary fibrosis; pulmonary arterialhypertension; vasculitis; pulmonary throm-boembolism; aspiration pneumoniaSerositis; occasional nephritis, cardiac dys-function; neurologic involvement

Differential diagnosisLupus erythematosus; dermatomyositis;progressive systemic sclerosis

TherapySevere involvement with evidence of organdysfunction: prednisone�; steroid sparingagents: cyclosporine; azathioprine; cyclo-phosphamide

ReferencesFarhey Y, Hess EV (1997) Mixed connective tissue

disease. Arthritis Care & Research 10(5):333–342

Mixed cryoglobulinemia


Mixed porphyria

� Variegate porphyria

Moeller's disease


Mole

� Nevus, melanocytic

Möller-Barlow disease


Molluscum



Synonym(s)Water wart; molluscum; molluscum seba-ceum; epithelioma contagiosum

DefinitionViral skin infection that produces papulesand nodules

PathogenesisCaused by large DNA poxvirus, Mollus-cipoxvirus; replicate in the cytoplasm ofepithelial cells and produce cytoplasmicinclusions and enlargement of infected cells

Clinical manifestationSolitary or grouped, asymptomatic, firm,smooth, umbilicated papules, on the skinand mucosal surfaces; may coalesce into

Molluscum contagiosum. Crystalline papules with central dell on the face


392 Molluscum sebaceum

plaques; self-limited, but sometimes per-sists for months to years; multiple, wide-spread, persistent lesions occurring inimmunocompromised patients, particu-larly those with HIV disease

Differential diagnosisWart; nevocellular nevus; varicella; fibrouspapule of the face; basal cell carcinoma;sebaceous gland hyperplasia; xanthoma;milia; syringoma; juvenile xanthogranu-loma; epidermoid cyst; granuloma annu-lare; cryptococcosis; histoplasmosis

TherapyCryotherapy; curettage; tretinoin; benzoylperoxide; disseminated disease in immuno-compromised patients: cidofovir 0.3% gelapplied twice daily for 7–14 days

ReferencesSmith KJ, Skelton H (2002) Molluscum contagio-

sum: recent advances in pathogenic mecha-nisms, and new therapies. American Journal of Clinical Dermatology 3(8):535–545

Molluscum sebaceum


Mometasone furoate


Mondor’s disease

Synonym(s)Subcutaneous phlebitis of the breast andchest wall; sclerosing periphlebitis of thelateral chest wall

DefinitionThrombophlebitis of the superficial veins ofthe anterior chest wall

PathogenesisUnknown

Clinical manifestationPain and tenderness of lateral chest walland/or breast, followed within hours todays by subcutaneous cord, with skinretraction

Differential diagnosisBreast carcinoma; breast abscess; foreignbody reaction; insect bite reaction

TherapyWarm compresses for symptomatic relief

ReferencesMayor M, Buron I, de Mora JC, Lazaro TE, Hern-

andez-Cano N, Rubio FA, Casado M (2000) Mondor's disease. International Journal of Der-matology 39(12):922–925

Mongolian spot

Synonym(s)Congenital dermal melanocytosis

DefinitionMacular, blue-gray pigmentation usually onthe sacral area and back of neonates

PathogenesisResults from arrested migration of melano-cytes in the dermis during migration fromthe neural crest into the epidermis

Clinical manifestationCongenital, asymptomatic, blue-gray, mac-ular hyperpigmentation, most commonlyinvolving the lumbosacral area, but alsobuttocks, flanks, and shoulders; mostlesions resolve in early childhood, but somepersist for many years


Morphea 393

M

Differential diagnosisNevus of Ota/Ito; blue nevus; child abuse


ReferencesMallory SB (1991) Neonatal skin disorders. Pedi-

atric Clinics of North America 38(4):745–761

Monilethrix

DefinitionBeaded pattern on the hair shaft

ReferencesLandau M, Brenner S, Metzker A (2002) Medical

pearl: an easy way to diagnose severe neonatal monilethrix. Journal of the American Academy of Dermatology 46(1):111–112

Moniliasis

� Candidiasis

Morbid hair pulling


Morphea

Synonym(s)Localized scleroderma; circumscribed scle-roderma

DefinitionDisorder characterized by skin and subcu-taneous tissue induration and thickeningdue to excessive collagen deposition

PathogenesisMultiple theories of causation, includingendothelial cell injury, autoimmune prob-lems, and dysregulation of collagen produc-tion

Clinical manifestationPoorly defined areas of nonpitting edema,with sclerosis developing as diseaseprogresses; skin surface becomes smoothand shiny, with loss of hair follicles anddecreased ability to sweat; after months toyears, skin softens and become atrophicGuttate variant: small, white, minimallyindurated papulesLinear variant: discrete, indurated, linear,hypopigmented, sclerotic bandsFrontoparietal linear morphea (en coup desabre): linear, atrophic plaque, suggestive ofa stroke from a sword, sometimes eventuat-ing in hemifacial atrophyProgressive hemifacial atrophy (Romberg-Perry syndrome): primary lesion occurringin the subcutaneous tissue, muscle, andbone; dermis affected only secondarily andskin not scleroticEosinophilic fasciitis: involves primarily thefascia; characterized by acute onset of pain-ful, indurated skin, usually of the upperextremity, with orange-peel appearance andswelling of the affected extremityDiffuse variant: widspread hypopigmented,sclerotic plaques, often involving the uppertrunk, abdomen, buttocks, and thighs

Differential diagnosisLichen sclerosus, necrobiosis lipoidica;granuloma annulare; graft versus host dis-ease; porphyria cutanea tarda; hyper-trophic scar; progressive systemic sclerosis;mixed connective tissue disease; lipoder-matosclerosis; phenylketonuria; radiationfibrosis; scleromyxedema; Werner syn-drome; medication- or chemical-inducedscleroderma

TherapyLocalized disease: no effective therapy; dif-fuse or symptomatic disease: photother-apy; physical therapy; prednisone; plas-


394 Morquio syndrome

mapheresis; D-penicillamine: 2.5 mg per kgPO daily

ReferencesHawk A, English JC 3rd (2001) Localized and sys-

temic scleroderma. Seminars in Cutaneous Medicine & Surgery 20(1):27–37

Morquio syndrome

Synonym(s)Mucopolysaccharidosis type IV-A

DefinitionInherited metabolic storage disease arisingfrom a deficiency of N-acetylgalactosamine-6-sulfatase (type IV-A) or beta-galactosi-dase deficiency (type IV-B)

PathogenesisAutosomal recessive trait; deficiency of N-acetylgalactosamine-6-sulfatase, resultingin accumulation of keratan sulfate (type IV-A) or beta-galactosidase deficiency(type IV-B), leading to accumulation ofchondroitin-6-sulfate (type IV-B) in theconnective tissue, the skeletal system, andthe teeth

Clinical manifestationAbnormalities of the skeletal system (e.g.,kyphoscoliosis, pectus carinatum, luxationof the hips); aortic valvular disease; dental

abnormalities; odontoid hypoplasia, withsubsequent atlantoaxial instability; hearingdeficit; diffuse corneal opacification andalterations of the trabecular meshwork;occasional glaucoma; type IV-B: hearingdeficits, dental abnormalities; cardiac mur-murs; hepatomegaly; no joint laxity

Differential diagnosisHurler syndrome; Hunter syndrome; Gau-cher’s disease; Niemann-Pick diseae; osteo-genesis imperfecta

TherapyInvestigational enzyme replacement withgalactose-6-sulfatase

ReferencesNorthover H, Cowie RA, Wraith JE (1996) Muco-

polysaccharidosis type IVA (Morquio syn-drome): a clinical review. Journal of Inherited Metabolic Disease 19(3):357–365

Mortification

� Gangrene

Morve


Mosaic speckled lentiginous nevus

� Nevus spilus

Mucha-Habermann disease

� Pityriasis lichenoides

Morphea. Sclerotic, white plaques on the hand


Mucormycosis 395

M

Mucocutaneous lymph node syndrome

� Kawasaki disease

Mucopolysaccharidosis type I-H

� Hurler syndrome

Mucopolysaccharidosis type I-H/S

� Scheie syndrome

Mucopolysaccharidosis type I-S

� Scheie syndrome

Mucopolysaccharidosis type II

� Hunter syndrome

Mucopolysaccharidosis type III-A

� Sanfilippo syndrome

Mucopolysaccharidosis type III-B


Mucopolysaccharidosis type III-C


Mucopolysaccharidosis type IV-A

� Morquio syndrome

Mucormycosis

Synonym(s)Phycomycosis; zygomycosis

DefinitionInfection with fungi of the order Mucor-ales, of which Rhizopus species are themost common causative organisms, thataffects otherwise chronically ill or immuno-supressed patients

PathogenesisInhalation of airborne mucorales spores,which settle in sinuses or lungs; local exten-sion, lymphatic, or hematogenous spreadfrom original site; invasion of blood vesselwalls, thrombosis, and infarction producesigns and symptoms of disease

Clinical manifestationCutaneous variant: secondary infection inburns or other trauma


396 Mucosal neuroma syndrome

Superficial variety: occurs in healthy peo-ple after trauma; vesicles, pustules, andplaquesGangrenous variant: solitary, violaceous,painful, papule or plaque, with ecchymoticcenter; may ulcerate and disseminate;occurs in immunosuppressed patientsRhinocerebral variant: progressive orbitalswelling and facial cellulitis, with dischargeof black pus from the necrotic palatine ornasal eschars; proptosis; chemosis; ophthal-moplegia; blindness; decreased conscious-ness suggests spread to brain; non-specificgastrointestinal and pulmonary signs andsymptoms

Differential diagnosisAspergillosis; nocardiosis; anthrax; orbitalcellulitis; pseudallescheria boydii infection;disseminated Fusarium infection; ecthymagangrenosum

TherapyAmphotericin B�: 1–1.5 mg per kg IV dailyinfused over 4–6 hours

ReferencesEucker J, Sezer O, Graf B, Possinger K (2001) Mu-

cormycoses. Mycoses 44(7-8):253–260

Mucosal neuroma syndrome

Synonym(s)MEN III syndrome; MEN IIB syndrome;multiple mucosal neuroma syndrome;Sipple syndrome

DefinitionOne of the multiple endocrine neoplasia(MEN) syndromes, characterized by tumorsof neuroendocrine origin

PathogenesisAutosomal dominant trait; gene mutationson chromosome 10

Clinical manifestationOral mucosal neuroma: yellow-white, ses-sile, painless papule of the lips, anteriortongue, and buccal commissures; similarlesions seen on the eyelids, sometimes pro-ducing eversion of the lid, and on the sclera;facial skin, especially around the nose,sometimes involved; lesions develop in firstdecade of life; tall, lanky, marfanoid bodytype, with a narrow face and muscle wast-ing; adrenal and thyroid tumors presentafter puberty; associated with adrenal phe-ochromocytoma, medullary thyroid carci-noma, diffuse alimentary tract ganglioneu-romatosis, and multiple, small, submucosalneuroma nodules of the upper aerodiges-tive tract

Differential diagnosisGranular cell tumor; neurofibroma;fibroma; squamous cell carcinoma; Gard-ner’s syndrome; tuberous sclerosis

TherapySurgical removal for esthetic purposes or ifrepeatedly traumatized

ReferencesLee NC, Norton JA (2000) Multiple endocrine ne-

oplasia type 2B-genetic basis and clinical ex-pression. Surgical Oncology 9(3):111–118

Mucosal pemphigoid

� Cicatricial pemphigoid

Mucosal sebaceous cysts


Mucous cyst



Multicentric reticulohistiocytosis 397

M

Mud fever

� Leptospirosis

Muir-Torre syndrome

Synonym(s)Torre syndrome

DefinitionFamilial cancer syndrome consisting of atleast one sebaceous neoplasm (sebaceousadenoma, sebaceous epithelioma, or seba-ceous carcinoma) and at least one visceralmalignancy, usually gastrointestinal or gen-itourinary carcinoma

PathogenesisAutosomal dominant trait involving muta-tions in mismatched repair genes, mostlythe MSH2 gene, located on chromosomearm 2p

Clinical manifestationOne or more sebaceous neoplasms, includ-ing sebaceous adenoma, sebaceous epitheli-oma, or sebaceous carcinoma, often on theface; other cutaneous neoplasms includekeratoacanthoma, squamous cell carci-noma, and multiple follicular cysts; one ormore visceral malignancies, most com-monly colorectal cancer or genitourinarymalignancies, either preceding or followingthe sebaceous tumors

Differential diagnosisGardner syndrome; Cowden syndrome;multiple trichoepitheliomas; basal cellnevus syndrome; basal cell carcinoma;squamous cell carcinoma; eruptive keratoa-canthomas; tuberous sclerosis

TherapySurgical excision of sebaceous neoplasms�;isotretinoin as prophylactic agent

ReferencesOmura NE, Collison DW, Perry AE, Myers LM

(2002) Sebaceous carcinoma in children. Jour-nal of the American Academy of Dermatology 47(6):950–953

Multicentric reticulohistiocytosis

Synonym(s)Lipoid dermatoarthritis; lipoid rheuma-tism; giant cell reticulohistiocytosis

DefinitionDisorder characterized by dermal papulesand nodules consisting of histiocytic prolif-eration, associated with arthritis

PathogenesisMay be a paraneoplastic response to under-lying malignancy in some cases

Clinical manifestationAsymptomatic to slightly pruritic, skin-colored to reddish-brown papules or nod-ules, usually on the upper potion of thebody; may be isolated from one another ormay be clustered, sometimes giving a cob-blestone appearance; polyarthritis may pre-cede or follow onset of skin lesions; remis-sion may occur after years

Differential diagnosisRheumatoid nodule; xanthoma; dermatofi-broma; progressive nodular histiocytoma;xanthoma; juvenile xanthogranuloma; lep-rosy; granuloma annulare; Jessner’s lym-phocytic infiltration; lupus erythematosus;Langerhans cell histiocytosis; lipogranulo-matosis; gouty tophi; sarcoidosis; osteoar-thritis, psoriatic arthritis, Reiter disease

TherapyPrednisone; triamcinolone, intralesional;hydroxychloroquine; methotrexate; photo-chemotherapy


398 Multiple hamartoma syndrome

ReferencesRapini RP (1993) Multicentric reticulohistiocyto-

sis. Clinics in Dermatology 11(1):107–111

Multiple hamartoma syndrome

� Cowden disease

Multiple hemangiomata syndrome


Multiple idiopathic hemorrhagic sarcoma

� Kaposi’s sarcoma

Multiple lentigines syndrome


Multiple mucosal neuroma syndrome


Multiple symmetrical lipomatosis


Mupirocin

Trade name(s)Bactroban

Generic availableNo

Drug classTopical antibiotic

Mechanism of actionSelective binding to bacterial isoleucyltransfer-RNA synthetase, causing inhibi-tion of protein synthesis

Dosage form2% cream, ointment


Common side effectsCutaneous: burning sensation, dryness,pruritus; redness

Serious side effectsCutaneous: superinfection after prolongeduse


Mupirocin. Dermatologic indications and dosage


Impetigo Apply 3 times daily for 7–14 days Apply 3 times daily for 7–14 days


Mycetoma 399

M

Contraindications/precautionsHypersensitivity to drug class or compo-nent; caution when using in large openwounds

ReferencesWilliford PM (1999) Opportunities for mupirocin

calcium cream in the emergency department. Journal of Emergency Medicine 17(1):21Ï–220

Murrain


Musculoaponeurotic fibromatosis

� Desmoid tumor

Mycetoma

Synonym(s)Madura foot; maduromycosis

DefinitionChronic granulomatous disease of the skinand subcutaneous tissue, characterized bytumefaction, abscess formation, and fistu-lae

PathogenesisCaused by true fungi (eumycetoma) or byaerobic bacterial actinomycetes (actino-mycetoma)Organisms producing eumycetoma: Pseu-dallescheria boydii (the most commoncause in the United States); Madurella myc-etomatis; Madurella grisea; Phialophorajeanselmei; Pyrenochaeta romeroi; Lept-

osphaeria senegaliensis; Curvularia lunata;Neotestudina rosatii; Aspergillus nidulans;Aspergillus flavus; species of Fusarium:Cylindrocarpon and AcremoniumOrganisms causing actinomycetoma: Actin-omadura madurae and A. pelletieri; Strep-tomyces somaliensis; several species andvarieties of Nocardia, particularly N. brasil-iensis; organisms introduced via localizedtrauma of the skin with thorns, wood splin-ters, or implantation with solid objects

Clinical manifestationOccurs most commonly in people that workin rural areas where they are exposed toacacia trees or cactus thorns containing theetiologic agents; slow-growing, painless,suppurative papules and nodules, abscessesand fistulae drain clear, viscous, or puru-lent exudate or grains; affects upper andlower limbs, particularly the feet and lowerlegs; progressive extension and formationof multiple sinus tracts; extensive tissueswelling, induration, and destruction;chronic lesions contain healed, scarred,sometimes closed sinus tracts with new,open, suppurative tracts in other adjacentareas; invasion of bone cortex results inreplacement of osseous tissues and marrowby masses of grains

Mycetoma. Multiple, infiltrated nodules on the foot


400 Mycobacterium marinum infection

Differential diagnosisSporotrichosis; coccidioidomycosis; tuber-culosis; osteogenic neoplasms; osteomyeli-tis; botryomycosis

TherapyEumycetoma: ketoconazole, itraconazole;surgical excision if no response to medicaltherapyActinomycetoma: trimethoprim-sulfameth-oxazole, with or without amikacin, 15 mgper kg per day IM; dapsone




Synonym(s)Fish tank granuloma; swimming pool gran-uloma; fish fancier's finger

DefinitionAtypical mycobacterial infection followingskin trauma in fresh or salt water, charac-terized by localized granuloma or sporotri-chotic lymphangitis

PathogenesisCaused by inoculation by Mycobacteriummarinum, occurring following trauma toskin in contact with an aquarium, saltwater, or marine animals

Clinical manifestationAfter 2–3 week incubation period, papule orbluish nodule appears at inoculation site,with subsequent ulceration; new lesionsmay occur along path of lymphatic drain-age

Differential diagnosisOther atypical mycobacterial pathogens,such as M. chelonae, M. fortuitum, or M.

gordonae; bacterial pyoderma; herpeticwhitlow; sporotrichosis; nocardiosis; inocu-lation coccidioidomycosis; orf; milker’snodule; cutaneous tuberculosis; anthrax;listeriosis; leishmaniasis; squamous cellcarcinoma; foreign body granuloma

TherapyClarithromycin; minocycline; cipro-floxacin; trimethoprim-sulfamethoxazole;surgical hyperthermia; surgical excision

ReferencesAubry A, Chosidow O, Caumes E, Robert J, Cam-

bau E (2002) Sixty-three cases of Mycobacteri-um marinum infection: clinical features, treatment, and antibiotic susceptibility of caus-ative isolates. Archives of Internal Medicine 162(15):1746–1752

Mycobacterium ulcerans infection

� Buruli ulcer

Mycophenolate mofetil

Trade name(s)CellCept

Generic availableNo

Drug classImmunosuppressive

Mechanism of actionInhibits T-cell and B-cell proliferation byblocking de novo purine synthesis; non-competitive inhibitor of inosine monophos-phate dehydrogenase



Myiasis 401

M


Common side effectsCardiovascular: peripheral edemaGastrointestinal: diarrhea, abdominal pain,nausea and vomitingGenitourinary: urinary urgency, fre-quency, and dysuriaHematologic: leukopeniaLaboratory: hypokalemia, hypercholestero-lemiaNeurologic: headachePulmonary: cough

Serious side effectsGastrointestinal: bleeding, ulceration, orperforationHematologic: bone marrow suppression,immunosuppressionInfectious: susceptibility to infection,malignancy

Drug interactionsAcyclovir; azathioprine; oral contracep-tives; ganciclovir; iron salts; probenecid

Contraindications/precautionsHypersensitivity to drug class or compo-nent; pregnancy; caution in patients with

severe renal or gastrointestinal disease; cau-tion with bone marrow suppression

ReferencesKitchin JE, Pomeranz MK, Pak G, Washenik K,

Shupack JL (1997) Rediscovering mycophenolic acid: a review of its mechanism, side effects, and potential uses. Journal of the American Academy of Dermatology 37(3 Pt.1):445–449

Mycosis fungoides

� T-cell lymphoma, cutaneous

Myiasis

Synonym(s)None

DefinitionInvasion of living tissue by the larvae (mag-gots) of two-winged flies (Diptera)

Mycophenolate mofetil. Dermatologic indications and dosage


Bullous pemphigoid 1–1.5 gm PO twice daily 600 mg per m2 PO twice daily


1–1.5 gm PO twice daily 600 mg per m2 PO twice daily

Pemphigus foliaceus, including fogo selvagem


Pemphigus vulgaris 1–1.5 gm PO twice daily 600 mg per m2 PO twice daily

Psoriasis 1–1.5 gm PO twice daily 600 mg per m2 PO twice daily



Reiter syndrome 1–1.5 gm PO twice daily 600 mg per m2 PO twice daily


1–1.5 gm PO twice daily Not applicable


402 Myoepithelioma

PathogenesisFly eggs deposited on the skin; larvae feedon wound debris, penetrate skin, and causeinflammatory response

Clinical manifestationWound variant: superficial inflammatoryreaction on surface; furuncular (follicular)variant: larvae penetrate skin; pruriticinflammatory papule with volcano-like cen-tral punctum; intermittent sanguineous orserosanguineous discharge

Differential diagnosisTungiasis; furuncle; infected epidermoidcyst; insect bite reaction; foreign bodygranuloma; atypical mycobacterial infec-tion; anthrax; nocardia infection; leishma-niasis

TherapySurgical excision; lidocaine injectionbeneath furuncle, then push organism intothe punctum.; superficial incision followedby gentle pressure, inward and downward;bacon fat applied adjacent to the punctum;petroleum jelly applied over punctum

ReferencesSampson CE, MaGuire J, Eriksson E (2001) Botfly

myiasis: case report and brief review. Annals of Plastic Surgery 46(2):150–152

Myoepithelioma


Myosarcoma

� Rhabdomyosarcoma

Myxedema

DefinitionNon-pitting edema of the skin due to infil-tration of the subcutaneous tissues by met-achromatic proteoglycans in patients withhypothyroidism

ReferencesGuha B, Krishnaswamy G, Peiris A (2002) The di-

agnosis and management of hypothyroidism. Southern Medical Association Journal 95(5):475–480

Myxedematosus


Myxoid cyst


Myxedema. Minimally infiltrated plaque on the anterior leg


Myxomatous degenerative cyst 403

M

Myxoma-spotty pigmentation-endocrine overactivity

� Carney’s syndrome

Myxomatous cutaneous cyst


Myxomatous degenerative cyst



N

Naegeli-Franceschetti-Jadassohn syndrome

Synonym(s)

None

Definition

Form of ectodermal dysplasia character-ized by reticulate pigmentation and kerato-derma

Pathogenesis

Autosomal dominant trait; possibly associ-ated with markers located near the type Ikeratin gene


Reticulate hyperpigmentation beginning atage 1–5 years and improving after puberty;palmar and plantar hyperkeratosis withlack of dermatoglyphics; hypohidrosis withheat intolerance, worsened by reducedsweating; dental abnormalities includingdefective dentures with yellow spots on theenamel


Incontinentia pigmenti; X-linked reticulatepigmentary disorder; dermatopathia pig-mentosa reticularis; Dowling-Degos dis-ease; confluent and reticulated papillomato-sis of Gougerot and Carteaud; reticulatedacropigmentation of Kitamura; hereditarybullous acrokeratotic poikiloderma ofWeary-Kindler; acromelanosis progressiva;

dyschromia universalis hereditaria; hid-rotic ectodermal dysplasia; hereditary bul-lous acrokeratotic poikiloderma

Therapy


References

Schnur RE, Heymann WR (1997) Reticulate hy-perpigmentation. Seminars in Cutaneous Med-icine & Surgery 16(1):72–80

Naevus a pernione

�

Angiokeratoma of Mibelli

Naevus maternus

�

Nevus flammeus

Naftifine

Trade name(s)

Naftin

Generic available

No


406 Nail biting

Drug class

Allylamine antifungal agent

Mechanism of action

Inhibition of squalene epoxidase, with sub-sequent reduction of cell wall ergosterolsynthesis

Dosage form

1% cream; 1% gel


See table

Common side effects

Cutaneous:

burning sensation, pruritus,erythema, dryness


None

Drug interactions

None



References

Muhlbacher JM (1991) Naftifine: a topical al-lylamine antifungal agent. Clinics in Dermatol-ogy 9(4):479–485

Nail biting

�

Onychophagia

Nail-patella syndrome

Synonym(s)

Hereditary osteo-onychodysplasia

;

Fongdisease

;

onychoosteodysplasia

;

Turner-Kieser syndrome

Definition

Hereditary disorder characterized by fin-gernail dysplasia, absent or hypoplasticpatellae, posterior conical iliac horns,deformation or luxation of the radial heads,and occasional nephropathy

Pathogenesis

Autosomal dominant trait; gene located onchromosome 9 at locus linked to that of theABO blood group adenylate kinase andlocus of the alpha 1 chain of type 5 colla-gen; altered connective tissue metabolismwith widespread structural defects in colla-gen; abnormal collagen deposition in theglomeruli may cause nephropathy


Nail changes: absent or short nail plate; V-shaped triangular lunulae with a distal peakin the midline; other abnormalities include:splitting, longitudinal ridging, koilonychia,poor lunula formation, and discolorationSkeletal changes: small or absent patella;elbows may have limited motion; subluxa-tion of the radial head; arthrodysplasia ofthe elbows; hyperextension of the joints;exostosesKidney changes: usually only asympto-matic proteinuria, but hematuria, neph-rotic syndrome, and renal failure may occur

Naftifine. Dermatologic indications and dosage


Cutaneous candidiasis Apply daily for 3–6 weeks Apply daily for 3–6 weeks

Tinea capitis Apply daily for 3–6 weeks Apply daily for 3–6 weeks

Tinea corporis Apply daily for 3–6 weeks Apply daily for 3–6 weeks

Tinea cruris Apply daily for 3–6 weeks Apply daily for 3–6 weeks

Tinea pedis Apply daily for 3–6 weeks Apply daily for 3–6 weeks


Necrobiotic xanthogranuloma 407

N


Pachonychia congenita; other congenitalectodermal defects

Therapy

No effective therapy for skin defects

References

Ogden JA, Cross GL, Guidera KJ, Ganey TM (2002) Nail patella syndrome. A 55-year follow-up of the original description. Journal of Pedi-atric Orthopaedics, Part B 11(4):333–338

Nakagawa’s angioblastoma

�

Tufted angioma

Nakagawa’s angioma

�

Tufted angioma

NAME syndrome

�

Carney’s syndrome

Necrobiosis lipoidica

Synonym(s)

Necrobiosis lipoidica diabeticorum

Definition

Localized disorder of collagen, with con-nective tissue degeneration, granuloma-tous reaction, thickening of blood vesselwalls, and deposition of fat

Pathogenesis

Theories of causation: microangiopathy;trauma; metabolic derangement; antibody-mediated vasculitis


Well-circumscribed papule or nodule withactive border, usually over pretibial area,but sometimes arising on face, trunk, orextremities; evolves into waxy, atrophic,round plaque beginning with red-browncolor but progressing to yellow-browncolor; painful ulcerations after weeks tomonths


Morphea; lichen sclerosus; nodular vasculi-tis; Weber-Christian disease; factitial dis-ease; granuloma annulare; sarcoidosis;necrobiotic xanthogranuloma; xanthoma

Therapy

Corticosteroids, topical, super potent; tri-amcinolone 3–4 mg per ml intralesional;tretinoin; aspirin/dipyrimidine; pentoxifyl-line: 400 mg PO 3 times daily

References

Sibbald RG, Landolt SJ, Toth D (1996) Skin and di-abetes. Endocrinology & Metabolism Clinics of North America 25(2):463–472

Necrobiosis lipoidica diabeticorum

�

Necrobiosis lipoidica

Necrobiotic xanthogranuloma

Synonym(s)

None


408 Necrolytic migratory erythema

Definition

Inflammatory histiocytic granulomatosis,characterized by slowly enlarging papulesand plaques

Pathogenesis

Associated with paraproteinemia and cry-oglobulinemia in some cases; associatedwith myeloma


Asymptomatic, firm, red-to-orange papulesor nodules, coalescing into plaques thatmay ulcerate; lesions become yellowish asthey evolve; located on face, trunk orextremities; hepatosplenomegaly; arthropa-thy


Necrobiosis lipoidica; granuloma annulare;xanthoma; multicentric reticulohistiocyto-sis; squamous cell carcinoma; atypicalfibroxanthoma

Therapy

Prednisone; radiation therapy; chloram-bucil: 2 mg PO daily; plasmapheresis

References

Mehregan DA, Winkelmann RK (1992) Necrobiot-ic xanthogranuloma. Archives of Dermatology 128(1):94–100

Necrolytic migratory erythema

�

Glucagonoma syndrome

Necrotic arachnidism

�


Necrotizing erysipelas

�

Necrotizing fasciitis

Necrotizing fasciitis

Synonym(s)

Hospital gangrene

;

acute infective gan-grene

;

necrotizing erysipelas

;

suppurativefasciitis

Definition

Bacterial soft tissue infection characterizedby fascial necrosis

Pathogenesis

Subcutaneous bacterial invasion causesdecreased oxygen tension, which reducesneutrophil function; multiple bacterialpathogens, including: Group A beta-hemo-lytic streptococci (most common organ-ism), Haemophilus aphrophilus, Staphylo-coccus aureus, Clostridium perfringens andsynergistic anaerobic and facultative bacte-ria; bacterial superantigens produceextreme immunologic response and subse-quent tissue injury


Most commonly involves extremities ortrunk, but may involve perineum (Fornier’s

Necrotizing fasciitis.

Necrotic plaque with bullae in the groin area


Nephrogenic fibrosing dermopathy 409

N

gangrene); often follows trauma, surgicalwound or hematogenous seeding fromanother site; early, severe, local pain, out ofproportion to visible findings; poorly mar-ginated red plaque with subcutaneousedema, which progresses to dusky plaquewith vesiculation and occasional crepitus;marked constitutional changes, includingfever, prostration, decreased sensorium,and hypotension


Cellulitis; polyarteritis nodosa or other vas-culitides; insect envenomation; pyodermagangrenosum; acute febrile neutrophilicdermatosis; vascular insufficiency

Therapy

Emergency surgical debridement

�

;penicillin G 8–10 million units per day IV,given every 4–6 hours; clindamycin

References

Levine N, Kunkel M, Nguyen T, Ackerman L (2002) Emergency Department Dermatology. Current Problems in Dermatology 14(6):183–220

Necrotizing glomerulonephritis

�


Necrotizing granulomatous inflammation of the respiratory tract

�


Necrotizing livedo reticularis

�

Calciphylaxis

Necrotizing lymphadenitis

�

Kikuchi’s syndrome

Necrotizing lymphocytic folliculitis

�

Acne necrotica

Neonatal pustular melanosis

�

Transient neonatal pustular mela-nosis

Nephrogenic fibrosing dermopathy

Synonym(s)

Scleromyxedema-like illness of hemodialy-sis

;

scleromyxedema-like illness of renaldisease

Definition

Disorder of patients with renal disease,characterized by thickening and hardeningof the skin

Pathogenesis

Involves mucin deposition in the skin


Thickening and hardening of skin, mostcommonly over extremities and trunk,while sparing face; hyperpigmentation insclerotic areas; flexion contractures; firm,yellowish papules and nodules; occurs inchronic renal failure, during hemodialysisor after renal transplantation


410 Netherton syndrome


Scleromyxedema; progressive systemic scle-rosis; morphea; porphyria cutanea tarda;eosinophilic fasciitis; eosinophilia-myalgiasyndrome; toxic oil syndrome; amyloidosis

Therapy


References

Streams BN, Liu V, Liegeois N, Moschella SM (2003) Clinical and pathologic features of ne-phrogenic fibrosing dermopathy: a report of two cases. Journal of the American Academy of Dermatology 48(1):42–47

Netherton syndrome

Synonym(s)

Netherton’s syndrome

;

Còmel-Nethertonsyndrome

Definition

Hereditary syndrome characterized by con-genital erythroderma, trichorrhexis invagi-nata, ichthyosis linearis circumflexa, atopicdiathesis, and failure to thrive

Pathogenesis

Autosomal recessive trait, with gene locali-zation to chromosome 5q32; intermittentkeratinizing defect of the hair cortex result-ing from incomplete conversion of sulfhy-dryl –SH group into S-S disulfide bonds inthe protein of the cortical fibers, whichcauses cortical softness, bulging, and bam-boo deformity


Congenital erythroderma; bamboo hairabnormality (trichorrhexis invaginata),leading to sparse, short, spiky, lusterless,and brittle hair; intermitent serpiginousmigratory annular/polycyclic eruption withdouble-edged scale (ichthyosis linearis cir-cumflexa), lasting for weeks to months;atopic diathesis, with multiple food aller-gies; early failure to thrive, with diarrhea

and symptoms of malabsorption, whichimproves with age


Other causes of congenital erythroderma,including lamellar ichthyosis; erythrokera-toderma variabilis; acrodermatitis entero-pathica; seborrheic dermatitis; Leiner dis-ease

Therapy

Emollients; corticosteroids, topical, lowpotency

References

Siegel DH, Howard R (2002) Molecular advances in genetic skin diseases. Current Opinion in Pediatrics 14(4):419–25

Netherton’s syndrome

�

Netherton syndrome

Neurilemmoma

Synonym(s)

Benign schwannoma

;

neurinoma

;

neurolemmoma

;

perineural fibroblastoma

Definition

Benign nerve sheath tumor derived fromSchwann cells

Pathogenesis

Alteration or loss of the NF2 tumor sup-pressor gene may be partially responsiblefor tumor formation


Asymptomatic, slow-growing, solitary ormultiple, flesh-colored papules or nodules,with predilection for head, neck, and flexorsurfaces of the upper and lower extremi-ties; neurilemmomatosis (schwannomato-sis) variant: subset of neurofibromatosistype 2 (NF2); autosomal dominant disor-


Neurofibromatosis 411

N

der; multiple, encapsulated nodules, locatedin the subcutaneous tissue


Neurofibroma; neuroma; leiomyoma;myoblastoma; epidermoid cyst; lipoma

Therapy

Surgical excision

�

ReferencesSmith JT, Yandow SM (1996) Benign soft-tissue le-

sions in children. Orthopedic Clinics of North America 27(3):645–654

Neurinoma

� Neurilemmoma

Neurocysticercosis

� Cysticercosis

Neurodermatitis


Neurodermatitis circumscripta


Neurofibroma

� Neurofibromatosis

Neurofibromatosis

Synonym(s)Von Recklinghausen's disease; Von Reck-linghausen disease

DefinitionHereditary disorder with multiple pheno-types, affecting skin, bone, nervous system,and soft tissue, most characteristic findingof which is mulitple neurofibromas

PathogenesisAutosomal dominant trait, but many spon-taneous mutations; NF-1 variant: linked tolarge gene on band 17q11.2, which encodestumor suppressor protein, neurofibromin;NF-2 variant: mutation of unknown tumorsuppressor protein; segmental variant: maybe related to mosaicism or segmentalhyperexpression

Clinical manifestationNF-1 variant: 6 or more café au lait maculeslarger than 0.5 cm in prepubertal individu-als and those larger than 1.5 cm in postpu-bertal individuals; two or more neurofibro-mas of any type or 1 plexiform neurofi-broma; axillary freckling; optic glioma; irishamartomas (Lisch nodules); osseouslesionsNF-2 variant: 8th cranial nerve tumors; neu-rofibromas; meningiomas; gliomas;schwannomasSegmental variant: multiple soft papules(neurofibromas) in a nerve segment distri-bution

Differential diagnosisProteus syndrome; McCune-Albright syn-drome LEOPARD syndrome; Carney’s syn-drome; Watson syndrome; tuberous sclero-sis; Noonan’s syndrome

TherapySurgical excision of symptomatic tumors�


412 Neurofibromatosis with Noonan phenotype

ReferencesLynch TM, Gutmann DH (2002) Neurofibromato-

sis 1. Neurologic Clinics 20(3):841–865

Neurofibromatosis with Noonan phenotype

� Watson syndrome

Neurofibromatosis-Noonan syndrome

� Watson syndrome

Neurofollicular hamartoma

� Trichodiscoma

Neurolemmoma

� Neurilemmoma

Neurothekeoma

Synonym(s)Neurothekeoma of Gallager and Helwig;benign nerve sheath tumor; perineuralmyxoma

DefinitionBenign skin or mucous membrane tumor ofnerve sheath origin

PathogenesisUnknown

Clinical manifestationAsymptomatic, soft, flesh-colored-to-redpapule, usually on the face or proximalupper extremities, but occasionally in oralmucous membrane; appears in the first twodecades of life

Differential diagnosisDermal nerve sheath myxoma; neurofi-broma; neural nevus; schwannoma


ReferencesTomasini C, Aloi F, Pippione M (1996) Cellular

neurothekeoma. Dermatology 192(2):160–163

Neurothekeoma of Gallager and Helwig

� Neurothekeoma

Neutral lipid storage disease


Neutrophilic dermatitis

� Acute febrile neutrophilic dermatosis

Nevocellular nevus

� Nevus, melanocytic


Nevoxanthoendothelioma 413

N

Nevoid basal cell carcinoma syndrome


Nevoid hypermelanosis

Synonym(s)Lentiginous hyperpigmentation; linear andwhorled nevoid hypermelanosis

DefinitionCongenital disorder characterized bystreaks and whorls of macular hyperpig-mentation along Blaschko’s lines

PathogenesisPresumed to represent somatic mosaicism

Clinical manifestationOnset in first few weeks of life; irregularswirls of macular hyperpigmentation, fol-lowing Blaschko’s lines; may cross the mid-line and be discontinuous; may fade some-what as child ages

Differential diagnosisIncontinentia pigmenti; hypomelanosis ofIto; Nevus of Ota and Ito; post-inflamma-tory hyperpigmentation; nevus spilus


ReferencesSchepis C, Siragusa M, Alberti A, Cavallari V

(1996) Linear and whorled nevoid hypermela-nosis in a boy with mental retardation and con-genital defects. International Journal of Dermatology 35(9):654–655

Nevoid hypertrichosis

Synonym(s)Nevoid hypertrichosis; faun-tail nevus

DefinitionDisorder characterized by solitary or fewcircumscribed areas of terminal hairgrowth, which is abnormal in length, shaftdiameter, or color

PathogenesisUnknown

Clinical manifestationPatches of terminal hair growth, occurringanywhere from the neck or legs to thepalms; usually present at birth or in earlyinfancy; when present in lumbosacral area(faun-tail nevus), associated with underly-ing kyphoscoliosis or partial spinal cordduplication

Differential diagnosisBecker’s nevus; Cornelia de Lange syn-drome; congenital hemihypertrophy withhypertrichosis; hypertrichosis lanuginosa;hypertrichosis associated with neurologicdisorders


ReferencesChang SN, Hong CE, Kim DK, Park WH (1997) A

case of multiple nevoid hypertrichosis. Journal of Dermatology 24(5):337–341

Nevoxanthoendothelioma

� Juvenile xanthogranuloma


414 Nevus anemicus

Nevus anemicus

Synonym(s)None

DefinitionCongenital vascular anomaly, character-ized by a pale-colored patch resulting fromlocalized reduced blood flow

PathogenesisPharmacologic anomaly caused byincreased vascular sensitivity to catecho-lamines

Clinical manifestationPermanent, irregularly shaped, pale coloredpatch, with stellate margins; usually locatedon the upper trunk; present at birth, butsometimes difficult to discern because ofsimilarity of color to background; increasedfrequency in patients with neurofibromato-sis

Differential diagnosisNevus depigmentosus; hypomelanosis ofIto; segmental vitiligo; tinea versicolor;post-inflammatory hypopigmentation; lep-rosy; tuberous sclerosis


ReferencesAhkami RN, Schwartz RA (1999) Nevus anemi-

cus. Dermatology 198(4):327–329

Nevus araneus

� Spider angioma

Nevus, Becker’s


Nevus comedonicus

� Epidermal nevus

Nevus, connective tissue


Nevus depigmentosus

Synonym(s)Achromic nevus

DefinitionCongenital and stable localized area ofhypopigmentation or depigmentation

PathogenesisMay involve defective melanin transferfrom melanocytes to keratinocytes

Clinical manifestationPale-colored patch, with streaks, whorls;round in contour; no change with age

Differential diagnosisHypomelanosis of Ito; tinea versicolor;vitiligo; leprosy; nevus anemicus; post-inflammatory hypopigmentation; tuberoussclerosis


ReferencesPinto FJ, Bolognia JL (1991) Disorders of hypopig-

mentation in children. Pediatric Clinics of North America 38(4):991–1017


Nevus lipomatosis 415

N

Nevus flammeus

Synonym(s)Nevus flammeus neonatorum; port-winestain; port-wine mark; strawberry patch;naevus maternus

DefinitionCongenital malformation of the upper der-mal blood vessels producing a permanent,localized, red patch

PathogenesisDecreased local innervation may producedecreased vascular tone and progressivevascular dilatation

Clinical manifestationPink-to-violaceous patch, with variableblanching after external pressure; presentfrom birth; usually located over the headand neck area; surface sometimes becomesthickened with a cobblestone-like contourand vascular papules or nodules or pyo-genic granulomas, usually in adulthood;skin and underlying soft tissue or bonyhypertrophy may occur.Sturge-Weber (encephalofacial or encepha-lotrigeminal angiomatosis) variant: vascu-lar malformation involving the upper facialarea supplied by ophthalmic branch(CN V1) of the trigeminal nerve, the ipsilat-eral leptomeninges, and the ipsilateral cere-bral cortex; more extensive than in isolatednevus flammeus; complications includeglaucoma, seizures, hemiplegia, mentalretardation, cerebral calcifications, sub-dural hemorrhage, and underlying soft tis-sue hypertrophy

Differential diagnosisCapillary hemangioma; salmon patch;Beckwith-Wiedemann syndrome; Coatsdisease; Cobb syndrome; Parkes-Webersyndrome; phakomatosis pigmentovascula-ris; von Hippel-Lindau disease; Wyburn-Mason syndrome

TherapyFlashlamp-pumped pulse dye laser�

ReferencesTravelute Ammirati C, Carniol PJ, Hruza GJ

(2001) Laser treatment of facial vascular le-sions. Facial Plastic Surgery 17(3):193–201

Nevus flammeus neonatorum

� Nevus flammeus

Nevus fuscoceruleus acromiodeltoideus


Nevus fuscoceruleus ophtalmomaxillaris


Nevus fuscoceruleus zygomaticus


Nevus lipomatosis

Synonym(s)Nevus lipomatosis of Hoffmann-Zurhelle;nevus lipomatosus cutaneous superficialis


416 Nevus lipomatosis of Hoffmann-Zurhelle

DefinitionDisorder characterized by solitary orgrouped hamartomatous proliferations offatty tissue

PathogenesisUnknown

Clinical manifestationAsymptomatic, soft, skin colored to yellowpapules and nodules, which often coalesceinto plaques; surface is either smooth,wrinkled, cerebriform, or verrucoid, withcomedones; distribution usually linear, sys-tematized, zosteriform, or along the lines ofskin folds, with predilection for the pelvicgirdle, lumbar area, buttocks, and the upperthighs; solitary type consists of papule ornodule with no favored location, usuallyappearing during the third to sixth decadesof life

Differential diagnosisFocal dermal hypoplasia; lipoma; epider-mal nevus, melanocytic nevus; nevus seba-ceous; skin tags; connective tissue nevus;accessory nipple; neurofibroma; angiol-ipoma; trichoepithelioma; cylindroma;localized scleroderma

TherapySurgical excision for cosmesis only

ReferencesIoannidou DJ, Stefanidou, M P, Panayiotides, JG,

Tosca, A D (2001) Nevus lipomatosus cutane-ous superficialis (Hoffmann-Zurhelle) with lo-calized scleroderma like appearance. International Journal of Dermatology 40(1):54–57

Nevus lipomatosis of Hoffmann-Zurhelle

� Nevus lipomatosis

Nevus lipomatosus cutaneous superficialis

� Nevus lipomatosis

Nevus, melanocytic

Synonym(s)Nevocellular nevus; mole

DefinitionBenign neoplasm composed of melanocytes

PathogenesisPropensity to develop multiple lesions, par-ticularly atypical moles; may be autosomaldominant trait; ultraviolet radiation may becofactor

Clinical manifestationCongenital variant: size ranging from <1 cmto lesions covering most of the integument;range in color from tan to deep blue-black;may begin as patch and become palpable aschild ages; associated satellite pigmentedpapules, especially in patients with giantcongenital nevus (>20 cm in diameter);melanoma risk increases with size of con-genital lesion

Nevus, melanocytic. Large, irregular hyperpigmented plaque over the trunk and buttocks


Nevus of Ota and Ito 417

N

Acquired variant: sharply marginated, uni-form tan to brown, smooth to verrucouspapule or macule, usually <1 cm in diame-terSpitz (spindle cell) nevus variant: uniform,smooth, reddish-brown papule, often withfine overlying scale, usually occurring inchildhoodBlue nevus variant: uniform, firm, bluepapuleClark’s nevus (atypical mole, dysplasticnevus) variant: reddish-brown flat papule,with central elevation and feathered redborder (“fried egg appearance”), often>0.5 cm in diameter; sometimes marker ofrisk for melanoma, particularly with familyhistory of melanoma or presence of multi-ple lesions

Differential diagnosisMelanoma; seborrheic keratosis; nevus ofOta and Ito; lentigo; freckle; mastocytoma;juvenile xanthogranuloma; basal cell carci-noma; actinic keratosis; benign tumor ofsweat gland or hair follicle

TherapyBiopsy of all lesions in which melanoma isin the differential diagnosis�; congenitalnevus: surgical excision, particularly largerlesions�; acquired nevus, blue nevus, orSpitz nevus: surgical excision for cosmesisonly; Clark’s nevus: controversial whethersurgical excision is indicated

ReferencesMakkar HS, Frieden IJ (2002) Congenital melano-

cytic nevi: an update for the pediatrician. Cur-rent Opinion in Pediatrics 14(4):397–403

Schaffer JV, Bolognia JL (2000) The clinical spec-trum of pigmented lesions. Clinics in Plastic Surgery 27(3):391–408

Nevus mucinosis


Nevus of Cannon


Nevus of Ito


Nevus of Jadassohn and Tieche

� Blue nevus

Nevus of Ota and Ito

Synonym(s)Nevus fuscoceruleus zygomaticus; Hori’snevus; Hori nevus; nevus fuscoceruleusacromiodeltoideus; oculodermal melano-sis; nevus fuscoceruleus ophtalmomaxil-laris; oculodermal melanocytosis

DefinitionMelanin pigmentation of the facial skin, thesclera of the eye, and the oral mucosa (Otavariant), or over the shoulder (Ito variant)

PathogenesisMay represent embryonic melanocytes thathave not migrated completely from the neu-ral crest to the epidermis

Clinical manifestationNevus of Ota: usually unilateral, poorlydemarcated, gray-blue patch over the cheek,forehead, eyelid, temple, and gingiva; sclera


418 Nevus of Sutton

blue and shiny; often follows distribution ofthe two first branches of the trigeminalnerve; sometimes slowly and progressivelyenlarges and darkens; usually stable onceadulthood reachedNevus of Ito: same appearance and courseas nevus of Ota, but located over shoulderand upper arm areas

Differential diagnosisBlue nevus; melasma; ochronosis;melanoma; lentigo; traumatic tattoo

TherapyQ-switched ruby, Q-switched alexandrite orQ-switched Nd:YAG laser

ReferencesMishriki YY (2001) Are these pigmentary changes

only cosmetic? Oculodermal melanocytosis (nevus of Ota). Postgraduate Medicine 110(6):43–46

Nevus of Sutton

� Halo nevus

Nevus on nevus

� Nevus spilus

Nevus sebaceous

� Epidermal nevus

Nevus simplex

� Salmon patch

Nevus spilus

Synonym(s)Speckled lentiginous nevus; mosaic speck-led lentiginous nevus; nevus on nevus;speckled nevus spilus

DefinitionLesions characterized by tan patches con-taining numerous darker macules orpapules

PathogenesisMay represent localized defect of melano-blast migration populating a particular areaof skin; mosaicism possible cause of zoster-iform variant

Clinical manifestationVariable number of black, brown, or red-brown macules and papules seen withinoval or linear (zosteriform) patch of tan tobrown hyperpigmentation, often present atbirth; some follow lines of Blaschko

Differential diagnosisCongenital nevus; Spitz nevus; NAME syn-drome; LEOPARD syndrome; Carney’s syn-drome

TherapySurgical excision for cosmesis only; Q-switched ruby or Q-switched Nd:YAG laserablation for cosmesis only

Nevus spilus. Speckled brown patch on the trunk


Niacinamide (nicotinamide) 419

N

ReferencesCarpo BG, Grevelink JM, Grevelink SV (1999) La-

ser treatment of pigmented lesions in children. Seminars in Cutaneous Medicine & Surgery 18(3):233–243

Nevus spilus tardus


Nevus syringadenoma papilliferum

� Syringocystadenoma papilliferum

Nevus unius lateris

� Epidermal nevus

Nevus varicousus osteohypertrophicus syndrome


Nevus verrucosus

� Epidermal nevus

Nevus verrucosus hypertrophicans


New World spotted fever

� Rocky Mountain spotted fever

Niacin deficiency

� Pellagra

Niacinamide (nicotinamide)

Trade name(s)None


Drug classVitamin

Mechanism of actionSuppression of antigen-induced lymphob-last transformation; mast cell stabilization



Common side effectsNeurologic: headache; dyspepsia

Serious side effectsGastrointestinal: hepatotoxicity




420 Nicholas Favre disease

ReferencesChaidemenos GC (2001) Tetracycline and niaci-

namide in the treatment of blistering skin dis-eases. Clinics in Dermatology 19(6):781–785001

Nicholas Favre disease


Niemann disease


Niemann's disease


Nikolsky sign

DefinitionCondition where the epidermal layer of skinslips free from the lower layers with a slightrubbing pressure

ReferencesNousari HC, Anhalt GJ (1999) Pemphigus and

bullous pemphigoid. Lancet 354(9179):667–672

Nocardia infection

� Nocardiosis

Nodose fever


Nodular cutaneous elastoidosis with cysts and comedones

� Favre-Racouchot syndrome

Nodular nonsuppurative panniculitis

� Weber-Christian disease

Niacinamide (nicotinamide). Dermatologic indications and dosage


Bullous pemphigoid 500 mg PO 2–3 times daily, often given concomitantly with tetracycline, doxycycline, or minocycline

500 mg PO 2–3 times daily, often given concomitantly with tetracycline, doxycycline, or minocycline




Pellagra 100 mg PO every 6 hours for several days or until major acute symptoms resolve, followed by 50 mg PO 2–3 times daily until skin lesions heal

100 mg PO every 6 hours for several days or until major acute symptoms resolve, followed by 50 mg PO 2–3 times daily until skin lesions heal


Noonan’s syndrome 421

N

Nodular subepidermal fibrosis

� Dermatofibroma

Nodular vasculitis

Synonym(s)Bazin’s disease; erythema induratum

DefinitionNodular eruption on the lower legs, withhistopathologic changes similar to ery-thema induratum (i.e., vasculitis of largervessels and panniculitis)

PathogenesisHypersensitivity reaction to endogenous orexogenous antigens, which include tuberclebacillus

Clinical manifestationChronic, recurrent crops of small, tender,erythematous nodules on the legs;depressed scars, or pigmentation from pre-viously active lesions

Differential diagnosisErythema nodosum; chilblains; T-cell lym-phoma; erythema nodosum leprosum; fac-titial panniculitis; panniculitis associatedwith alpha-1 antitrypsin deficiency; pancre-atic panniculitis; lupus panniculitis; super-ficial thrombophlebitis

TherapyAntituberculous therapy if associated withtuberculosis�; potassium iodide 300–500 mg PO three times daily; prednisone;bed rest

ReferencesPhelps RG, Shoji T (2001) Update on panniculitis.

Mount Sinai Journal of Medicine 68(4-5):262–267

Non-metastasizing fibrosarcoma

� Desmoid tumor

Non-venereal syphilis of children

� Bejel

Nonbullous congenital ichthyosiform erythroderma

� Lamellar ichthyosis

Noonan’s syndrome

Synonym(s)Familial Turner syndrome; female pseudoTurner syndrome; pseudo Turner syn-drome; male Turner syndrome; pseudo Ull-rich-Turner syndrome; Turner-like syn-drome; Ullrich-Noonan syndrome; Turnerphenotype syndrome

DefinitionFamilial syndrome characterized by shortstature, low-set ears, and many minor skele-tal deformities, of which the commonest arepectus excavatum and cubitus valgus

PathogenesisAutosomal dominant trait; unknown genedefect

Clinical manifestationShort stature; low set ears; skeletal anoma-lies, including pectus excavatum and cubi-tus valgus; intelligence often below aver-age; cardiac abnormalities including pul-


422 North American blastomycosis

monary valve stenosis, right heartanomalies, and left ventricular cardiomyop-athy

Differential diagnosisTurner’s syndrome; neurofibromatosis;edema neonatorum; Aarskog’s syndrome;Watson’s syndrome; LEOPARD syndrome;fetal alcohol syndrome

TherapyGrowth hormone therapy for short stature

ReferencesKelnar CJ (2000) Growth hormone therapy in

Noonan syndrome. Hormone Research 53 Sup-pl 1:77–81


Synonym(s)Blastomycosis

DefinitionEndemic systemic mycotic infection causedby the thermally dimorphic fungus, Blasto-myces dermatitidis

PathogenesisInhalation of the microconidia from themold form of B dermatitidis into the lungscauses infection; transition from mold formto yeast form after deposition in distal air-ways; in the absence of nonspecific hostdefense mechanisms, cells increases innumber in the lungs; subsequent lympho-hematogenous spread to the other organs;rarely occurs in skin after direct innocula-tion

Clinical manifestationUsually starts with pulmonary infection fol-lowed by cutaneous, osseous, genitouri-nary, or central nervous system involve-ment; skin findings: most common mani-festation of extrapulmonary disease; lesionsfavor exposed areas; minimally tender

papules or pustules evolve into purulent,verrucous, or ulcerative nodules or plaques,characterized by sharp and heaped-up bor-ders with centrally located granulation tis-sue and exudate; pulmonary findings: signsof acute pneumonia, including fever, nightsweats, wheezing and dyspnea; signs andsymptoms of chronic pneumonia last for 2–6 months, including weight loss, nightsweats, fever, cough, and chest pain; osteo-lytic bone lesions; prostatitis or epididymi-tis

Differential diagnosisBasal cell carcinoma; squamous cell carci-noma; pyoderma gangrenosum; keratoa-canthoma; wart; leishmaniasis; anthrax;coccidioidomycosis; nocardiosis; atypicalmycobacterial infection; cutaneous tuber-culosis; sarcoidosis

TherapyAmphotericin B: 0.7–1 mg per kg IV perday; total dose 1.5–2.5 g; itraconazole; keto-conazole

ReferencesBradsher RW (1997) Therapy of blastomycosis.

Seminars in Respiratory Infections 12(3):263–267

Notalgia paresthetica

Synonym(s)Paresthetic notalgia

DefinitionSensory neuropathy involving dorsal spinalnerves causing proxysmal pruritus of theupper back

PathogenesisForm of peripheral neuropathy of unknowncause

Clinical manifestationPruritus, pain, and/or paresthesia occur-ring principally between the scapulas,


Nummular eczema 423

N

sometimes attacking either side of the mid-line or posterolateral aspect of the shoul-der; onset in early to middle adulthood;sometimes persists for decades; hyperpig-mentation secondary to chronic rubbingand scratching; rare early clinical marker ofmultiple endocrine neoplasia type IIA

Differential diagnosisIntercostal neuralgia; thoracic outlet syn-drome; lichen amyloidosis; atopic neuro-dermatitis; post-herpetic neuralgia; xero-sis; contact dermatitis

TherapyCapsaicin; oxcarbazepine 300 mg PO twicedaily; titration of dose to effect; local nerveblock

ReferencesMassey EW (1998) Sensory mononeuropathies.

Seminars in Neurology 18(2):177–183

Nummular dermatitis

� Nummular eczema

Nummular eczema

Synonym(s)Nummular dermatitis; discoid eczema

DefinitionChronic disorder characterized by pruritic,coin-shaped papules and plaques

PathogenesisAssociated with xerosis, atopy, and venousstasis

Clinical manifestationPapules or vesicles that coalesce to formconfluent plaques on erythematous base;early lesions sometimes exudative andcrusted; secondary infection may occur;necessitating systemic antibiotics; olderlesions are dry, scaly, and excoriated fromscratching; lower extremities and dorsum ofhand most frequently affected areas

Differential diagnosisAtopic dermatitis; tinea corporis; psoriasis;stasis dermatitis; pityriasis lichenoides;contact dermatitis; lichen simplex chroni-cus

TherapyCorticosteroids, topical, high potency�;prednisone for severe flares

ReferencesAoyama H, Tanaka M, Hara M, Tabata N, Tagami

H (1999) Nummular eczema: An addition of se-nile xerosis and unique cutaneous reactivities to environmental aeroallergens. Dermatology 199(2):135–139


O

Obliterative calcific-thrombotic arteriolopathy

�

Calciphylaxis

OCA

�

Oculocutaneous albinism

Occupational acne

�

Chloracne

Ochroid mycetoma

�

Eumycetoma

Ochronosis

Synonym(s)

Alcaptonuria

;

alkaptonuria

;

homogentisicacid oxidase deficiency

Definition

Inherited metabolic disorder characterizedby blue-black discoloration of certain tis-sues, including ear cartilage and ocular tis-sue

Pathogenesis

Autosomal recessive trait; caused by defi-ciency of homogentisic acid; deficiencyresults in accumulation and deposition ofhomogentisic acid in cartilage, causing dif-fuse bluish-black pigmentation


Dark urine in diapers usual first sign of dis-ease; gray-black scleral pigmentation inconfiguration of small, dark rings; ear carti-lage discoloration with a grayish-blue hue,followed by structural changes with stiff-ness, contour irregularities, and calcifica-tion; discoloration on nasal tip, costochon-dral junctions, extensor tendons of thehands, cheeks, fingernails, and buccalmucosa; ochronotic arthropathy; ochrono-sis-like pigmentation as idiosyncratic reac-tion to application of hydroquinone or phe-nol


Argyria; medication-related hyperpigmen-tation; arsenical keratosis

Therapy


�

Alcaptonuria


426 Ocular albinism

References

Kneebone TS, Selner AJ (1995) Ochronosis and alkaptonuria. Case report and literature review. Journal of the American Podiatric Medical As-sociation 85(10):554–555

Ocular albinism

�



Synonym(s)

Albinism

;

OCA

Definition

Group of disorders characterized by anabnormality in melanin synthesis due todysfunction of melanocytes in the skin,eyes, and/or ears

Pathogenesis

Autosomal recessive disorders; mutation ingenes that regulate the process of melaninsynthesis and distribution by the melano-cyteOCA Type 1: mutation in the tyrosinasegeneOCA Type 2: mutation in the P geneOCA Type 3: mutation in the tyrosinase-related protein-1 (TRP-1) gene


Three forms:OCA1: complete absence of pigment in theskin, hair, and eyes; photophobia; reducedvisual acuity; and nystagmusOCA2: minimal to moderate pigment in theskin, hair, and eyes; in some patients, pig-mented freckles, lentigines, and/or nevi;photophobia; reduced visual acuity; andnystagmusOCA3: minimal pigment reduction in theskin, hair, and eyes; mild photophobia;reduced visual acuity; and nystagmus

Ocular albinism: ocular depigmentationand iris translucency; motor nystagmus;reduced visual acuity; fundus hypopigmen-tation


Hermansky-Pudlak syndrome; phenylke-tonuria; Chediak-Higashi syndrome; histi-dinemia; homocystinuria; Menkes steelyhair disease; Tietz syndrome; Prader-Willisyndrome; Angelman syndrome

Therapy

Sun protection with protective clothing andsunscreens; corrective lenses for visualimpairment

References

Carden SM, Boissy RE, Schoettker PJ (1998) Albi-nism: Modern molecular diagnosis. British Journal of Ophthalmology (2):189–195

Oculodermal melanocytosis

�


Oculodermal melanosis

�


Oculomandibulodyscephaly with hypotrichosis

�


Oculomandibulofacial syndrome

�



Onychogryphosis 427

O

Ofuji disease

�


Ofuji’s disease

�


Ofuji’s papuloerythroderma

�


Olmsted syndrome

Synonym(s)

Olmsted’s syndrome

;

pluriorificial kerato-sis of Olmsted

;

congenital palmoplantarand periorificial keratoderma

Definition

Congenital palmoplantar keratoderma withprogressive palmoplantar hyperkeratosisand periorificial hyperkeratotic papulesand plaques

Pathogenesis

Unknown


At birth, sharply demarcated keratoticplaques involving periorificial sites; slow,progressive palmar and plantar kerato-derma, producing flexion deformities andautoamputation


Acrodermatitis enteropathica; pachonychiacongenita; mutilating palmoplantar kerato-derma

Therapy

Acitretin

References

Kress DW, Seraly MP, Falo L, Kim B, Jegasothy BV, Cohen B (1996) Olmsted syndrome. Case re-port and identification of a keratin abnormali-ty. Archives of Dermatology 132(7):797–800

Olmsted’s syndrome

�

Olmsted Syndrome

Omnipen

�

Ampicillin

Onchocerciasis

�

Filariasis

Onychocryptosis

Definition

Ingrowing of the nail plate

References

Ikard RW (1998) Onychocryptosis. Journal of the American College of Surgeons 187(1):96–102

Onychogryphosis

Definition

Nail plate enlargement with increasedthickening and curvature


428 Onycholysis

References

Mohrenschlager M, Wicke-Wittenius K, Brockow K, Bruckbauer H, Ring J (2001) Onychogryph-osis in elderly persons: an indicator of long-standing poor nursing care? Report of one case and review of the literature. Cutis 68(3):233–235

Onycholysis

Definition

Separation of the nail plate from the under-lying nail bed at distal and lateral attach-ments

References

Mohrenschlager M, Wicke-Wittenius K, Brockow K, Bruckbauer H, Ring J (2001) Onychogryph-osis in elderly persons: an indicator of long-standing poor nursing care? Report of one case and review of the literature. Cutis. 68(3):233–235

Onychomadesis

Definition

Complete separation of nail plate fromunderlying nail bed

References

Tosti A, Piraccini BM (2000) Treatment of com-mon nail disorders. Dermatologic Clinics 18(2):339

Onychomycosis

Synonym(s)

Fungal nail infection

Definition

Fungal infection affecting the toenails orthe fingernails

Pathogenesis

Caused by 3 classes of fungi: dermato-phytes (usually Trichophyton rubrum),yeasts, and nondermatophyte molds;spread from plantar skin to underside ofnail via the hyponychium or distal lateralnail bed


Distal lateral subungual variant: thickenedand opacified nail plate, nail bed hyperk-eratosis, and onycholysis; endonyx variant:milky-white discoloration of the nail platewithout subungual hyperkeratosis or ony-cholysis.Superficial white variant: confined to thetoenails, with small, white speckled or pow-dery patches on the surface of the nail plate;nail is roughened and crumbles easilyProximal subungual variant: leukonychia inthe proximal nail foldCandidal infection: paronychia; onycholy-sis; hyperkeratosis of nail bed and inflam-mation of the nail fold in chronic mucocu-taneous disease


Psoriasis; pityriasis rubra pilaris; twentynail dystrophy; lichen planus; traumaticnail dystrophy; contact dermatitis; pacho-nychia congenita; Darier disease; nailpatella syndrome; melanoma; bacterial par-onychia; yellow nail syndrome; drug-relatednail dystrophy

Therapy

Terbinafine; itraconazole; griseofulvin; flu-conazole; ciclopirox nail lacquer; surgicalnail avulsion and matrixectomy by chemi-cal or mechanical means

References

Crawford F, Young P, Godfrey C, Bell-Syer SE, Hart R, Brunt E, Russell I (2002) Oral treat-ments for toenail onychomycosis: a systematic review. Archives of Dermatology 138(6):811–816


Oral cutaneous fistula 429

O

Onychoosteodysplasia

�

Nail-patella syndrome

Onychophagia

Definition

Compulsive biting or chewing of the nails

References

Wells JH, Haines J, Williams CL (1998) Severe morbid onychophagia: the classification as self-mutilation and a proposed model of main-tenance. Australian & New Zealand Journal of Psychiatry 32(4):534–545

Onychorrhexis

Definition

Superficial splitting of the free edge of thenail

References

Bodman MA. (1995) Miscellaneous nail presenta-tions. Clinics in Podiatric Medicine & Surgery 12(2):327–346

Onychoschizia

Definition

Splitting of the fingernails at the distal tip

References

Bodman MA (1995) Miscellaneous nail presenta-tions. Clinics in Podiatric Medicine & Surgery 12(2):327–346

Onychotillomania

Definition

Compulsive picking at fingernails and/ortoenails

References

Colver GB (1987) Onychotillomania. British Jour-nal of Dermatology 117(3):397–399


Synonym(s)

Orofacial fistula

;

intra-oral fistula

;

dentalabscess with sinus tract formation

;

dentalsinus

Definition

Dental periapical inflammation, with devel-opment of a fistulous tract exiting throughthe face or neck

Pathogenesis

Direct extension or continuity from anacute irreversible pulpitis spreading beyondthe apex of the tooth or an acute exacerba-tion of a chronic apical periodontitis orperiapical granuloma; often associated withpoor oral hygiene and trauma; bacteriasuch as Streptococcus mutans, Staphylococ-cus epidermidis, Staphylococcus aureus,and Porphyromonas, Actinomycoses,Bacteroides, and Fusobacterium speciesfound at the site of the fistula


Reddish-brown nodule sometimes exudingserous or purulent material; most com-monly involves the mandible and chinregion; site of fistulation sometimes distantfrom the intraoral infection site


Pyogenic granuloma; melanoma; squa-mous cell carcinoma; basal cell carcinoma;


430 Oral epithelial nevus

nocardiosis, sporotrichosis; South Ameri-can blastomycosis; granuloma faciale; lupuserythematosus; epidermoid cyst

Therapy

Penicillin

�

; amoxicillin; doxycycline; inci-sion and drainage often necessary; extrac-tion of the affected tooth; pulpotomy, orpulp removal and drainage; surgicalremoval of sequestered or necrotic bone

�

References

Fernandez JM, Metlich MA, Bravo JM, Freyre IC (1982) Oral-cutaneous fistula of dental origin. Journal of Oral & Maxillofacial Surgery 40(3):183–185

Oral epithelial nevus

�

White sponge nevus

Oral fibroma

�

Angiofibroma

Oral florid papillomatosis

�

Verrucous carcinoma

Oral hairy leukoplakia

�

Hairy leukoplakia

Orf

Synonym(s)

Contagious ecthyma

;

ecthyma contagio-sum

;

ecthyma infectiosum

;

contagious pus-tular dermatitis

;

sheep pox

Definition

Viral disease of goats and sheep that can betransmitted to humans and produce self-healing cutaneous nodules

Pathogenesis

Caused by DNA virus belonging to Parap-oxvirus genus; infection from contact withinfected animals, carcasses, or nonlivingmaterial


Small, firm, red-to-blue papule which formhemorrhagic, flat-topped pustule or bulla,with crust or central umbilication, on thefingers, hands, or forearms; resolves after30–40 days


Tularemia; anthrax; milker’s nodule; acutefebrile neutrophilic dermatosis; leishmania-sis; bacterial ecthyma; cutaneous tuberculo-sis; sporotrichosis; nocardiosis; squamouscell carcinoma; keratoacanthoma

Therapy

No medical therapy; surgical excision ordestruction by electrodesiccation andcurettage for persistent lesion

References

Huerter CJ, Alvarez L, Stinson R (1991) Orf: case report and literature review. Cleveland Clinic Journal of Medicine 58(6):531–534


Osler-Weber-Rendu syndrome 431

O

Organoid nevus

�

Epidermal nevus

Oriental sore


Orofacial fistula


Orofacial granulomatosis


Oroya fever

� Bartonellosis

Osler disease


Osler's disease


Osler-Weber-Rendu syndrome

Synonym(s)Hereditary hemorrhagic telangiectasia;Rendu-Osler syndrome; Osler's disease;Osler disease; heredofamilial angiomatosis;familial hemorrhagic angiomatosis

DefinitionHereditary disorder characterized by tel-angiectasia and recurrent epistaxis

PathogenesisMutation of the protein endoglin, a recep-tor for transforming growth factor beta,with a role in tissue repair and angiogen-esis; defects in the endothelial cell junc-tions, endothelial cell degeneration, andweakness of the perivascular connective tis-sue cause dilation of capillaries and post-capillary venules, manifested as tel-angiectases

Clinical manifestationTelangiectases, found on the oral mucosa,nasal mucosa, skin, and conjunctiva; pin-head-sized macules or barely palpablepapules, partially blanching with pressure;color ranges from bright red to violaceousto purple; face, lips and mouth, nares,tongue, ears, hands, chest, and feet mostcommonly affected sites; cyanosis and club-bing in patients with pulmonary arterio-venous malformations; stroke, brainabscess, or intracerebral hematoma; pulmo-nary arterio-venous malformations, tach-ypnea; cyanosis; clubbing; retinal tel-angiectasias and hemorrhages; gastrointes-tinal bleeding; arterio-venous fistulas of theliver

Differential diagnosisCREST syndrome; Louis-Bar syndrome;ataxia-telangiectasia; benign essential tel-angiectasia; rosacea; actinically damagedskin; dermatomyositis; Rothmund-Thom-son syndrome; scleroderma; Cockayne syn-drome; angiokeratoma corporis diffusum


432 Osmidrosis

TherapyND:YAG laser ablation of symptomatic vas-cular lesions; recurrent, uncontrollableepistaxis: septal dermoplasty; bleedingprophylaxis: estradiol: 0.6 mg PO per day orvia transdermal patch

ReferencesHaitjema T, Westermann CJ, Overtoom TT, Tim-

mer R, Disch F, Mauser H, Lammers JW (1996) Hereditary hemorrhagic telangiectasia (Osler-Weber-Rendu disease): new insights in patho-genesis, complications, and treatment. Ar-chives of Internal Medicine 156(7):714–719

Osmidrosis

� Bromhidrosis

Osteitis fibrosa cystica


Osteitis fibrosa disseminata


Osteogenesis imperfecta

Synonym(s)None

DefinitionGroup of heritable disorders of collagensynthesis characterized by fragile bones

PathogenesisAutosomal dominant trait; mutation in pro-tein that codes for pro-α1 and pro-α2 colla-

gen chains, producing both qualitative andquantitative collagen defects

Clinical manifestationType 1: blue sclera; in-utero fractures; mild-to-moderate bone fragility; kyphoscoliosis;hearing loss; premature arcus senilis; easybruising; short statureType 2: abnormal dentition; blue sclera;small nose; micrognathia; connective tissuefragility; short trunkType 3: abnormal dentition; sclera of varia-ble hue; in-utero fractures; limb shorteningand progressive deformities; triangularfacies with frontal bossing; pulmonaryhypertensionType 4: normal sclera; normal hearing;fractures beginning in infancy; mild angu-lation and shortening of long bones

Differential diagnosisTurner syndrome; Paget disease; osteopet-rosis; camptomelic dysplasia; achondrogen-esis type I; congenital hypophosphatasia;steroid-induced osteoporosis; battered childsyndrome; copper deficiency

TherapyIntravenous aminohydroxypropylidene forincreasing bone mineral density

ReferencesCole WG (2002) Advances in osteogenesis imper-

fecta. Clinical Orthopaedics & Related Re-search (401):6–16

Osteoma cutis

Synonym(s)None

DefinitionPresence of bone within the skin withoutpreexisting or associated lesion

PathogenesisUnknown


Oudtshoorn skin 433

O

Clinical manifestationSingle or multiple, extremely hard papules,plaques, or nodules, usually on face,extremities, scalp, digits, and subungualregions; seen in Albright hereditary osteod-ystrophy, fibrodysplasia ossificans hetero-plasia, and fibrodysplasia ossificans pro-gressiva

Differential diagnosisCalcinosis cutis; pilomatricoma; metasta-sis; cartilaginous skin tumors; foreign bodyreaction; gouty tophus; myositis ossificans;secondary ossification


ReferencesOrlow SJ, Watsky KL, Bolognia JL (1991) Skin and

bones. II. Journal of the American Academy of Dermatology 25(3):447–462

Ostiderm


Otitis externa

Synonym(s)External otitis; swimmer’s ear

DefinitionInflammation of the skin of the ear canal,characterized by pain, redness, swelling,and discharge

PathogenesisNo single causative agent; often a combina-tion of infection with bacterial pathogens(Pseudomonas species or other gram-nega-tive organisms, S. aureus) or fungi (cand-ida and aspergillus species) and irritation;

predisposing factors are moisture withmaceration and trauma to mucosa

Clinical manifestationPainful tragus when applying traction tothe pinna; edema and redness of ear canal;purulent or serous discharge; conductivehearing loss; cellulitis of the face or neck;lymphadenopathy; fungal infections result-ing in severe itch but less pain than bacte-rial infection; thick dischargeNecrotizing (malignant) otitis externa vari-ant: pain out of proportion to clinical find-ings; granulation tissue in the ear canal

Differential diagnosisOtitis media; foreign body in ear canal;squamous cell carcinoma of ear canal; earcanal trauma; erysipelas

TherapyNeomycin, polymyxin B, hydrocortisoneotic solution applied 4 times daily for 10–14days�; hydrocortisone and acetic acid oticsolution applied on cotton wick 4 timesdaily for 10–14 days; acetic acid 5% in alu-minium acetate solution applied on cottonwick 3–4 times daily until symptoms abate;prednisone for severe inflammation; surgi-cal debridement in individuals with necro-tizing (i.e., malignant) variant

ReferencesSander R (2001) Otitis externa: a practical guide

to treatment and prevention. American Family Physician 63(5):927-936, 941–942

Ota, nevus of


Oudtshoorn skin



P

Pachydermia verticis gyrata

�

Cutis verticis gyrata

Pachydermodactyly

Synonym(s)

None

Definition

Form of acquired digital fibromatosis, char-acterized by non-inflammatory bulbousswelling of the dorsal and lateral surfaces ofthe fingers at the level of proxymalphalanges and interphalangeal joints

Pathogenesis

Possible role of repeated trauma, some-times in the background of neurotic behav-ior


Asymptomatic, persistent, symmetricalswellings on the lateral and medial aspectsof fingers; second, third, and fourth digitsaffected bilaterally; almost always in men


Knuckle pad; post-traumatic callosity; for-eign-body granuloma; fibroma; infantiledigital fibromatosis; rheumatoid andpseudo-rheumatoid nodule; arthritis; pach-ydermoperiostosis; proteus syndrome

Therapy

Triamcinolone 3–5 mg per ml intralesional

References

Tompkins SD, McNutt NS, Shea CR (1998) Distal pachydermodactyly. Journal of the American Academy of Dermatology 38(2 Pt 2):359–362


Synonym(s)

Primary hypertrophic osteoarthropathy

;

idiopathic hypertrophic osteoarthropathy

;

Touraine-Solente-Gole syndrome

Definition

Syndrome characterized by digital club-bing and subperiosteal new bone forma-tion, associated with pain, polyarthritis,cutis verticis gyrata, seborrheic dermatitis,and hyperhidrosis

Pathogenesis

Autosomal dominant trait with variablepenetrance; unknown gene defect


Digital clubbing and/or paronychial thick-ening; coarse facial features similar to thoseof acromegaly; scleroderma-like thickeningand furrowing of the skin on the foreheadand cheeks, with leonine facies in advancedstages; cutis verticis gyrata; seborrheic der-matitis of the face and the scalp; palmo-


436 Pachyonychia congenita

plantar hyperhidrosis or generalized hyper-hidrosis with secondary dermatitis


Acromegaly; thyroid acropachy; psoriaticarthritis; secondary hypertrophic pulmo-nary osteoarthropathy

Therapy


References

Sinha GP, Curtis P, Haigh D, Lealman GT, Dodds W, Bennett CP (1997) Pachydermoperiostosis in childhood. British Journal of Rheumatology 36(11):1224–1227

Pachyonychia congenita

Synonym(s)

Jadassohn-Lewandowsky syndrome

;

poly-keratosis congenita

Definition

Hereditary palmoplantar keratodermacharacterized by keratoderma of the palmsand soles and thickened nail plates

Pathogenesis

Usually autosomal recessive trait; muta-tions in the genes encoding epidermalkeratinocyte keratins, specifically K6a, K6b,K16, and K17, which disrupt keratin fila-ment assembly


Jadassohn-Lewandowsky type (PC-1):present at birth or from early childhood;thickened, brown-to-gray nail plates withrough surface; usually affects all fingers;toenails sometimes also involved; thick-ened fingernails may extend into periun-gual tissue, causing paronychia; circum-scribed or diffuse hyperkeratoses of palmsand soles; follicular hyperkeratosis on theface and on extensor aspect of proximalextremities; leukokeratosis of oral mucosa

Jackson-Lawler type (PC-2): thickened nailplates and other features of PC-1 type; natalteeth; unruly hair


Psoriasis; pityriasis rubra pilaris; ony-chomycosis; Darier disease; epidermolysisbullosa; mucocutaneous candidiasis

Therapy

Acitretin

�

References

Su WP, Chun SI, Hammond DE, Gordon H (1990) Pachyonychia congenita: a clinical study of 12 cases and review of the literature. Pediatric Dermatology 7(1):33–38

Paddy-field foot

�

Immersion foot

Paget’s disease

Synonym(s)

Paget’s disease of the nipple and areola

;

Paget’s disease of the skin, apocrine type

;

eczematoid epitheliomatous dermatosis

;

malignant papillary dermatosis

;

intraepidermal adenocarcinoma

Definition

Form of ductal carcinoma of either thebreast (mammary Paget’s disease) or theanogenital axillary, or other skin site(extramammary Paget’s disease)

Pathogenesis

Mammary variant: underlying intraductalcarcinoma of the breast with retrogradeextension into the overlying epidermisthrough mammary duct epithelium; tumorcells derive from luminal lactiferous ductalepithelium of the breast tissueExtramammary variant: in most cases,arises as a primary cutaneous adenocarci-


Palmoplantar hyperhidrosis 437

P

noma; epidermis is infiltrated with neoplas-tic cells showing glandular differentiation;tumor cells sometimes originate from apo-crine gland ducts or from keratinocyticstem cells


Mammary variant: may occur after longhistory of an eczematous skin lesion in thenipple and adjacent areas; sharply demar-cated, scaly, red, crusted, and thickenedplaques on the nipple, spreading to the sur-rounding areolar areas; may have retrac-tion of the nipple or palpable nodules, indi-cating an underlying breast cancer; sero-sanguinous nipple dischargeExtramammary variant: chronic, pruriticeczematous lesions in the groin, genitalia,perineum, or perianal area; unilateral,sharply marginated plaque with peripheralerythema; erosion or scaling sometimesoccurs in mature lesions


Mammary variant: irritant contact dermati-tis; atopic dermatitis; fixed medicationreaction; nipple duct adenoma; erosive ade-nomatosis of the nipple; melanoma;Bowen’s diseaseExtramammary variant: Bowen’s disease;basal cell carcinoma; melanoma; candidia-sis; intertrigo; contact dermatitis; sebor-rheic dermatitis; psoriasis; lichen simplexchronicus

Therapy

Mammary variant: mastectomy and lymphnode clearance

�

Extramammary variant: Mohs micro-graphic surgery

�

; wide local excision; imiq-uimod

References

Fu W, Mittel VK, Young SC (2001) Paget disease of the breast: analysis of 41 patients. American Journal of Clinical Oncology 24(4):397–400

Mehta NJ, Torno R, Sorra T (2000) Extramam-mary Paget's disease. Southern Medical Associ-ation Journal 93(7):713–715

Paget’s disease, extramammary

�

Paget’s disease

Paget's disease of the nipple and areola

�

Paget’s disease

Paget's disease of the skin, apocrine type

�

Paget’s disease

Painful papule

�

Piezogenic papule

Palmar fasciitis

�


Palmoplantar fibromatosis

�


Palmoplantar hyperhidrosis

Definition

Excess sweating of the palms and soles


438 Palmoplantar keratoderma

References

Togel B, Greve B, Raulin C (2002) Current thera-peutic strategies for hyperhidrosis: a review. European Journal of Dermatology 12(3):219–223

Palmoplantar keratoderma

Definition

Pathologic condition characterized by dif-fuse or localized thickening of the stratumcorneum, sometimes part of a generalizedcondition or a disorder primarily involvingthe hands and feet

�

Keratosis palmaris et plantaris

References

Zemtsov A, Veitschegger M (1993) Keratodermas. International Journal of Dermatology 32(7):493–498

Palmoplantar keratoderma areata

�

Striate keratoderma

Palmoplantar keratoderma diffusa circumscripta

�


Palmoplantar keratoderma mutilans

�

Vohwinkel syndrome

Palmoplantar keratoderma striata

�

Striate keratoderma

Palmoplantar keratoderma with periodontitis

�


Palmoplantar pustulosis

�

Psoriasis

Panatrophy of Gowers

Synonym(s)

Gowers’ panatrophy

;

Gowers’ local panat-rophy

Definition

Disorder characterized by plaques of mor-phea-like, cutaneous atrophy due to partialor total loss of subcutaneous fat and atro-phy of overlying skin, sometimes associ-ated with atrophy or impaired growth ofunderlying muscle or bone

Pathogenesis

May be the end result of more than onepathologic process; reduced sympatheticskin response and aberrant production ofnon-esterified fatty acids after stimulationwith epinephrine in lesional skin


Sharply defined, irregular area of atrophy,developing over a period of a few weeks


Papillon-Lefèvre syndrome 439

P

without preceding inflammation; subcuta-neous fatty tissue regresses and overlyingskin appears atrophic, but otherwise nor-mal; atrophy reaches maximum extentwithin a few months and then stabilizesindefinitely


Sclerotic panatrophy; facial hemiatrophy(Romberg’s syndrome); morphea; pannicu-litis

Therapy


References

Sakamoto T, Oku T, Takagawa M (1998) Gowers' local panatrophy. Europeon Journal of Derma-tology 8(2):116–117

Panniculitis, cold

�

Cold panniculitis

Panniculitis of the newborn

�

Subcutaneous fat necrosis of new-born

Papillary adenoma of the nipple

�


Papillary hidradenoma

�

Hidradenoma papilliferum

Papillary intralymphatic angioendothelioma

�


Papillary syringadenoma

�

Syringocystadenoma papilliferum

Papillomatosis of the subareolar ducts

�



Synonym(s)

Palmoplantar keratoderma with periodon-titis

;

keratoderma palmoplantaris diffusawith periodontosis

Definition

Hereditary disorder characterized by pal-moplantar keratoderma and periodontosis

Pathogenesis

Autosomal recessive trait; gene locusmapped to 11q14-q21; possible dysfunctionof cathepsin C gene; possible defect in leu-kocyte function


Diffuse palmoplantar keratosis; scaly ery-thematous plaques over knees, elbows, andinterphalangeal joints; hyperhidrosis andmalodor; periodontosis with severe gingivi-


440 Papular acrodermatitis

tis and loss of teeth by age 5 years;increased susceptibility to infection


Olmsted syndrome; Richner-Hanhart syn-drome; Vohwinkel syndrome; mal deMeleda

Therapy

Acitretin

�

; aggressive dental care

References

Siragusa M, Romano C, Batticane N, Batolo D, Schepis C (2000) A new family with Papillon-Lefevre syndrome: effectiveness of etretinate treatment. Cutis 65(3):151–155

Papular acrodermatitis

�


Papular acrodermatitis of childhood

�


Papular angioplasia

�

Angiolymphoid hyperplasia with eosinophilia

Papular dermatitis of pregnancy

�


Papular infantile acrodermatitis

�


Papular mucinosis

Synonym(s)


;

myxedematosus

;

scleromyxedema

Definition

Spectrum of disease characterized by gen-eralized, densely grouped, dome-shapedpapules with increased mucin deposition inthe dermis, sometimes associated with amonoclonal gammopathy

Pathogenesis

May be a fibroblast disorder, causingincreased mucin deposition in the skin


Papular mucinosis (lichen myxedemato-sus) variant: dome-shaped and flesh-colored or erythematous papules, often in apattern of parallel ridges, sometimes coa-lescing into grouped lichenoid papules, ondorsal hands, face, or extensor surfaces ofthe arms and legs; with extensive involve-ment, leonine faces and difficulty openingthe mouthScleromyxedema variant: widespread, ery-thematous, indurated skin resembling scle-roderma, with diffuse tightness anddecreased range of motion; systemic mani-festations include restrictive and obstruc-tive pulmonary dysfunction, cardiovascu-lar abnormalities, and polyarthritis; gas-trointestinal symptoms (most commonlydysphagia) related to deficient esophagealperistalsis; proximal muscle weakness, pol-yarthritis; organic brain syndrome; ectro-


Paradoxical fibrosarcoma 441

P

pion and corneal opacities; cardiovascularabnormalities

Differential diagnosisPersistent acral papular mucinosis; malig-nant atrophic papulosis; scleroderma; lym-phoma; scleredema; leprosy; sarcoidosis;follicular mucinosis; Darier disease;Grover’s disease; colloid milium; granu-loma annulare; lipoid proteinosis; progres-sive nodular histiocytosis

TherapyAcetretin; prednisone; orthovoltage radia-tion; electron beam radiation; photochemo-therapy; plasmapheresis; extracorporealphotophoresis; dermabrasion; carbon diox-ide laser ablation

ReferencesRongioletti F, Rebora A (2001) Updated classifica-

tion of papular mucinosis, lichen myxedemato-sus, and scleromyxedema. Journal of the American Academy of Dermatology 44(2):273–281

Papular urticaria

Synonym(s)Insect bite reaction

DefinitionPruritic eruption occurring in some chil-dren after insect bites, most often fromfleas, chiggers, or mosquitoes

PathogenesisMay be autosensitization response toarthropod bite

Clinical manifestationSmall, firm, red, pruritic papules oftenappearing in tight clusters and lasting for2–12 days, at sites of insect bites; few newbites may cause reaction in old bite sites;post-inflammatory hyperpigmentation

Differential diagnosisUrticaria; mastocytosis; acral papular der-matitis of childhood; drug eruption; der-matitis herpetiformis; scabies; atopic der-matitis; prurigo nodularis

TherapyIce water soaks; insect repellants for proph-ylaxis

ReferencesHoward R, Frieden IJ (1996) Papular urticaria in

children. Pediatric Dermatology 13(3):246–249

Papulopustular facial dermatitis


Papulosis atrophicans maligna


Papulovesicular acrolocated syndrome


Paracoccidioidomycosis


Paradoxical fibrosarcoma

� Atypical fibroxanthoma


442 Parakeratose brilliante

Parakeratose brilliante


Paraneoplastic acrokeratosis

Synonym(s)Acrokeratosis paraneoplastica of Bazex;acrokeratosis paraneoplastica

DefinitionScaly acral papules, paronychia, nail dystro-phy, and keratoderma as signs of upper air-way and upper digestive tract cancer

PathogenesisPossible circulating antibodies to tumorantigens

Clinical manifestationStage 1: eruption confined to fingers andtoes, nasal bridge, and tips of ears; red,scaly papules; tender nail folds; nail platedystrophyStage 2: palms and soles scaly and red; hon-eycomb-like thickening of palms and fin-gers; facial eruption involving the cheeksand entire earStage 3: eruption extends to the proximalextremities; diffuse scalp scaling

Differential diagnosisContact dermatitis; lupus erythematosus;dermatomyositis; photosensitivity reac-tion; medication reaction

TherapyTreatment of the underlying neoplasm; nospecific therapy for cutaneous disease

ReferencesBolognia JL (1995) Bazex syndrome: Acrokerato-

sis paraneoplastica. Seminars in Dermatology 14(2):84–89


Synonym(s)None

DefinitionDisorder characterized by oral erosions andbullous skin lesions in patients with under-lying neoplastic disease

PathogenesisTumor antigens evoke immune response toplakins, molecules found in desmosomesand hemidesmosomes playing key role inintermediate filament attachment; targetantigens: desmoplakins I and II, bullouspemphigoid antigen I (BP230 kd or BPAG1),envoplakin, periplakin, and HD1/plectin

Clinical manifestationOral erosions or ulcerations, occurring any-where in the mouth, usually as first sign ofdisease; similar lesions in nose, pharynx,tonsils, gastrointestinal tract, respiratorytract, genital mucosal surfaces; variableskin eruptions include diffuse erythema,vesiculobullous lesions, papules, scalyplaques, exfoliative erythroderma, ero-sions, or ulcerations; ocular involvementvaries from conjunctivitis to symblepharonwith corneal scarring; most common asso-ciated malignancy: non-Hodgkin’s lym-phoma; others: chronic lymphocytic leuke-mia, Castleman tumor, giant cell lym-phoma, Waldenström macroglobulinemia,thymoma, bronchogenic squamous cell car-cinoma, and follicular dendritic cell sar-coma

Differential diagnosisErythema multiforme; Stevens-Johnsonsyndrome; toxic epidermal necrolysis; pem-phigus vulgaris; bullous pemphigoid; cica-tricial pemphigoid; epidermolysis bullosaacquisita; lichen planus


Paronychia 443

P

TherapyPrednisone; steroid-sparing drugs: azathio-prine; cyclosporine; mycophenolate mofetil;cyclophosphamide; plasmapheresis

ReferencesKimyai-Asadi A, Jih MH (2001) Paraneoplastic

pemphigus. International Journal of Dermatol-ogy 40(6):367–372

Parangi

� Yaws

Parapsoriasis

Synonym(s)None

DefinitionGroup of cutaneous diseases characterizedby scaly plaques having a resemblance topsoriasis

ReferencesLambert WC, Everett MA (1981) The nosology of

parapsoriasis. Journal of the American Acade-my of Dermatology 5(4):373–395

Parapsoriasis en plaque


Parapsoriasis guttata


Paratyphoid fever

� Salmonellosis

Paresthetic notalgia

� Notalgia paresthetica

Parinaud oculoglandular syndrome

� Bartonellosis

Parinaud's oculoglandular syndrome

� Bartonellosis

Parkes-Weber syndrome


Paronychia

Synonym(s)Finger infection; runaround abscess;fingernail infection; runaround infection

DefinitionSoft-tissue infection in the area around fin-gernail


444 Paroxysmal nocturnal hemoglobinuria

PathogenesisBreakdown of protective barrier betweennail plate and nail fold; entry of organismsinto nail crevice allow bacterial or fungalcolonization; acute variant: Staphylococcusaureus most common organism; chronicvariant: Candida albicans most commonpathogen; other causes: bacterial, mycobac-terial, or viral infection; metastatic cancer;subungual melanoma; squamous cell carci-noma

Clinical manifestationAcute variant: history of minor trauma ornail manipulation; pain, tenderness, andswelling in lateral nail fold; erythematous,edematous distal finger, sometimes withpurulent exudate, most prominent in proxi-mal and lateral nail fold area, with exten-sion into eponychium; purulence of the nailbed; onycholysisChronic variant: inflammation, pain, andswelling occur episodically, often afterexposure to moist environment; edema-tous, erythematous, tender nail folds with-out fluctuance; thickened and discolorednail plates, with transverse ridges

Differential diagnosisMucocutaneous candidiasis; herpetic whit-low; contact dermatitis; periungual wart;squamous cell carcinoma; melanoma; ony-chomycosis

TherapyAcute variant: warm water soaks; amoxicil-lin; surgical incision and drainage if abscessforms; chronic variant: avoidance of incit-ing factors such as exposure to moist envi-ronments or skin irritants; avoidance of nailmanipulation; if Candida is causative, topi-cal clotrimazole and/or fluconazole; inrecalcitrant cases, eponychial marsupializa-tion

ReferencesRockwell PG (2001) Acute and chronic parony-

chia. American Family Physician 63(6):1113–1116

Paroxysmal nocturnal hemoglobinuria

Synonym(s)Marchiafava-Micheli syndrome; Strübing-Marchiafava-Micheli syndrome

DefinitionClinical manifestation of red cell break-down with release of hemoglobin into theurine manifested by dark-colored urine inthe morning

PathogenesisGenetic mutation leading to inability tosynthesize glycosyl-phosphatidylinositol(GPI) anchor that binds proteins to cellmembranes; deficient hematopoiesis fromdiminished blood cell production withhypoplastic bone marrow

Clinical manifestationAnemia associated with cola-colored urine;venous thrombosis: vein thrombosis mani-fested as raised, painful, red papules andnodules affecting large areas, subsidingwithin a few weeks, occasionally withnecrosis and ulceration; hepatic veinthrombosis resulting in Budd-Chiari syn-drome; abdominal vein thrombosis produc-ing upper abdominal pain; cerebral veinthrombosis causing headache, papille-dema, or pseudotumor cerebri

Differential diagnosisSeptic vasculitis; leukemia cutis; lym-phoma; Wegener’s granulomatosis; polyar-teritis nodosa; cryoglobulinemia; Sweetsyndrome; pyoderma gangrenosum

TherapyThrombotic complications: heparin emer-gently; then maintenance with an oral anti-coagulant, such as warfarin; severe disease:bone marrow transplantation

ReferencesPackman CH (1998) Pathogenesis and manage-

ment of paroxysmal nocturnal haemoglobinu-ria. Blood Reviews 12(1):1–11


Pediculosis 445

P

Partial albinism

� Piebaldism

Partial albinism with immunodeficiency

� Griscelli syndrome

Paru

� Yaws

Pasini and Pierini, atrophoderma of

� Atrophoderma of Pasini and Pierini

Pathergy

DefinitionErythematous papule, >2 mm, at the pricksite 48 hours after superficial penetrationwith sterile needle

ReferencesLee LA (2001) Behcet disease. Seminars in Cuta-

neous Medicine & Surgery 20(1):53–57

Pattern baldness


Pearly penile papules

� Angiofibroma

Peat moss disease

� Sporotrichosis

Pediculosis

Synonym(s)Lice; phthiriasis

DefinitionInfestation with lice

PathogenesisThree types of human lice all belonging toorder Anoplura; body lice infest clothing,laying their eggs on fibers in the fabricseams; head and pubic lice infest hair, lay-ing eggs at base of hair fibers; organismstake blood meals by piercing host skin

Clinical manifestationPediculosis capitis (head lice): organismsmost commonly found in retroauricularscalp; nits attach to hair shafts just abovelevel of the scalp; pruritus with evidence ofexcoriation, particularly on the upper neckPediculosis corporis (body lice): nits foundin the seams of clothing, not on body ofhost; hemosiderin-stained purpuric spotswhere lice have fed (maculae ceruleae)Pediculosis pubis (pubic lice): lice and nitsvisible throughout pubic hair, extendingonto adjacent hair-bearing areas; sameorganism also infests eyelashes

Differential diagnosisHair casts; seborrheic dermatitis; scabies;impetigo; benign pigmented purpura; fol-liculitis decalvans; acne keloidalis


446 Pediculosis capitis

TherapyPermethrin 1% cream rinse�; complete nitremoval with nit comb or chemical removersuch as Step 2

ReferencesRoberts RJ (2002) Clinical practice. Head lice.

New England Journal of Medicine 346(21):1645–1650

Pediculosis capitis

� Pediculosis

Pediculosis corporis

� Pediculosis

Pediculosis palpebrum

� Pediculosis

Pediculosis pubis

� Pediculosis

Pellagra

Synonym(s)Niacin deficiency; vitamin B3 deficiency

DefinitionDisease caused by a deficient diet or failureof the body to absorb niacin or tryptophan,characterized by photosensitive dermatitis,diarrhea, dementia, and ultimately death ifuntreated

PathogenesisLate stage of severe and prolonged niacindeficiency, vitamin required for adequatecellular function and metabolism as anessential component in coenzyme I andcoenzyme II, which either donate or accepthydrogen ions in vital oxidation-reductionreactions; primary disease: inadequate nic-otinic acid (i.e., niacin) and/or tryptophanintake in diet; secondary disease: adequateamounts of niacin present in the diet, butother diseases or conditions interfere withabsorption and/or processing, such aschronic diarrhea, carcinoid syndrome, orHartnup syndrome

Clinical manifestationCutaneous findings: symmetrical areas ofinvolvement including dorsal surfaces ofhands, face, neck (Casal necklace), arms,and feetEarly skin changes: edematous, exudativeplaques, evolving to erythema on dorsa ofhands, with pruritus and burning sensa-tion; erythema sometimes evolves to cinna-mon brown in color; coalescent bullae insome patients; dry brown scales and crusts,resulting from hemorrhage, scale, and ery-thema on sun-exposed skinLate skin changes: darkly pigmented, thick-ened, dry, scaly, hard, rough, and crackedskin; glossitis with soreness of the mouthGastrointestinal findings: poor appetite;nausea; vomiting; diarrhea; epigastric dis-comfort; abdominal pain; increased saliva-tionNeuropsychiatric changes: headache, irrita-bility; poor concentration; anxiety; delu-sional state; hallucinations; stupor; apathy;tremor; ataxia; spastic paresis

Differential diagnosisDrug reaction; polymorphous light erup-tion; lupus erythematosus; erythropoieticprotoporphyria; porphyria cutanea tarda;variegate porphyria; contact dermatitis;actinic reticuloid; leprosy; Hartnup syn-drome

TherapyNiacinamide�


Pemphigus foliaceus 447

P

ReferencesHendricks WM (1991) Pellagra and pellagralike

dermatoses: etiology, differential diagnosis, dermatopathology, and treatment. Seminars in Dermatology 10(4):282–292

Pemphigoid

� Bullous pemphigoid

Pemphigoid gestationis


Pemphigoid vegetans


Pemphigus circinatus


Pemphigus erythematosus

� Pemphigus foliaceus

Pemphigus foliaceus

Synonym(s)Superficial pemphigus

DefinitionAutoimmune skin disorder characterizedby formation of superficial blisters in nor-mal-appearing skin

PathogenesisIgG (mainly IgG4 subclass) autoantibodiesdirected against desmoglein 1 (160 kDa),expressed mainly in the granular layer ofthe epidermis; medications and sunlightexposure may be precipitating factors

Clinical manifestationTransient, superficial vesicles and bullae,transforming into crusted or scaly erodedplaques on an erythematous base, mainly inseborrhoic areas, with little or no involve-ment of mucous membranes; pemphiguserythematosus (Senear-Usher) variant: fea-tures of cutaneous lupus erythematosusand pemphigus foliaceus; red scaly plaqueson the bridge of the nose and malar area;exfoliative erythroderma with extensiveinvolvement; pemphigus herpetiformis var-iant: pruritic grouped papules and vesicles,suggestive of dermatitis herpetiformis;occasional oral erosions; drug-induced var-iant: may occur with penicillamine or cap-topril therapy, usually after at least2 months of use; relatively mild signs andsymptoms

Differential diagnosisPemphigus vulgaris; paraneoplastic pem-phigus; bullous pemphigoid; erythemamultiforme; dermatitis herpetiformis; lin-

Pemphigus foliaceus Scaly, eroded plaques on the face


448 Pemphigus neonatorum

ear IgA dermatosis; lupus erythematosus;impetigo; Darier disease; transient acantho-lytic dermatosis; Hailey-Hailey disease;subcorneal pustular dermatosis

TherapyCorticosteroids, topical, super potent; pred-nisone; hydroxychloroquine; minocycline;steroid sparing agents: azathioprine; dap-sone; cyclophosphamide

ReferencesHuilgol SC, Black MM (1995) Management of the

immunobullous disorders. II. Pemphigus. Clinical & Experimental Dermatology 20(4):283–293

Pemphigus neonatorum

� Staphylococcal scalded skin syn-drome

Pemphigus paraneoplastica

� Paraneoplastic pemphigus

Pemphigus vegetans

� Vegetans pemphigus

Pemphigus vulgaris

Synonym(s)None

DefinitionAutoimmune blistering disease character-ized by superficial vesicles and bullae of theskin and mucous membranes

PathogenesisMediated by circulating autoantibodiesdirected against keratinocyte cell surfaceantigens, desmoglein 1 and desmoglein 3,which may have direct effect on desmo-somal function or may trigger cellular proc-ess resulting in acantholysis; may occur inpatients with other autoimmune diseases,particularly myasthenia gravis and thy-moma

Clinical manifestationMucous membrane lesions: painful, ill-defined, irregularly shaped, gingival, buc-cal, or palatine erosions; erosions some-times spread to larynx with subsequenthoarseness; other sites of mucous mem-brane involvement: conjunctiva, esopha-gus, labia, vagina, cervix, penis, urethra,and anusSkin lesions: fragile, flaccid vesicle or bullafilled with clear fluid, arising on normalskin or on an erythematous base; large ero-sions with lateral spread of blistersVegetating (vegetans) variant: lesions inskin folds form vegetating plaques withexcessive granulation tissue and crusting;occur more frequently in intertriginousareas and on scalp and face

Differential diagnosisPemphigus foliaceus; paraneoplastic pem-phigus; bullous pemphigoid; erythemamultiforme; dermatitis herpetiformis; Hai-

Pemphigus vulgaris. Eroded papules and plaques on the lip, face, and trunk


Penicillin VK 449

P

ley-Hailey disease; aphthous stomatitis;herpetic stomatitis; erosive lichen planus

TherapyPrednisone�; steroid sparing agents: aza-thioprine; dapsone; cyclophosphamide;mycophenolate mofetil; cyclosporine;auranofin; corticosteroids, topical, superpotent; intravenous immunoglobulin(IVIG): 2 gm IV divided over 3 days every4–8 weeks

ReferencesToth GG, Jonkman MF (2001) Therapy of pemphi-

gus. Clinics in Dermatology 19(6):761–767

Pemphigus vulgaris chronicus


Penicillin G benzathine

Trade name(s)Bicillin LA



Mechanism of actionInhibits penicillin-binding proteins, whichcause inhibition of bacterial cell wall syn-thesis

Dosage form300,000 units per ml; 600,000 units per mlfor intramuscular injection


Common side effectsCutaneous: urticaria and other skin erup-tionsGastrointestinal: nausea, vomiting, diarrhea

Serious side effectsBone marrow: thrombocytopeniaCutaneous: anaphylaxisGastrointestinal: pseudomembranous coli-tisRenal: interstitial nephritis


Contraindications/precautionsHypersensitivity to drug class or compo-nent; caution in patients with cepha-losporin allergy, seizure disorder, impairedrenal function



Penicillin VK

Trade name(s)Pen-Vee K; Veetids



Mechanism of actionInhibits penicillin-binding proteins, whichcause inhibition of bacterial cell wall syn-thesis

Dosage form250 mg, 500 mg tablets; 125 mg per 5 ml sus-pension; 250 mg per 5 ml suspension


450 Penile fibromatosis


Common side effectsCutaneous: urticaria and other skin erup-tionsGastrointestinal: nausea, vomiting, diarrhea

Serious side effectsBone marrow: thrombocytopeniaCutaneous: anaphylaxis, Stevens-Johnsonsyndrome, toxic epidermal necrolysisGastrointestinal: pseudomembranous coli-tisRenal: interstitial nephritis


Contraindications/precautionsHypersensitivity to drug class or compo-nent; caution in patients with cepha-losporin allergy, seizure disorder, impairedrenal function



Penile fibromatosis

� Peyronie’s disease

Penicillin G benzathine. Dermatologic indications and dosage


Bejel 2.4 million units IM (over age 10 years)

600,000 units IM as single injection


1.2 million units IM as single injection

0.3–0.6 million units IM for children < 27 kg; 0.9 million units IM for children > 27 kg

Erysipelas 1.2 million units IM as single injection

< 30 kg 300,000 units IM daily for 10–14 days

Impetigo 1.2 million units IM as single injection

0.3–0.6 million units IM for children < 27 kg; 0.9 million units IM for children > 27 kg

Pinta 2.4 million units IM (over age 10 years)

50,000 units IM as single dose

Scarlet fever 1.2 million units IM as single injection

300,000–600,00 units IM as single injection

Syphilis (primary, secondary, early latent)

2.4 million units IM (over age 10 years)

0.05 million units per kg IM weekly for 3 weeks; neonates > 1200 gm – 0.05 million units per kg IM for 1 dose

Tropical phagedenic ulcer

800,000–1,000,000 million units IM for a total dose of 6–10 million units

400,000–600,000 million units IM daily for a total dose of 3–5 million units

Yaws 1.2 million units IM as single injection

600,000 units IM as single injection


Perforating folliculitis 451

P

Penile lichen sclerosus


Perforating folliculitis

Synonym(s)Acquired perforating dermatosis; acquiredperforating dermatitis

DefinitionDisease characterized by disruption of theinfundibular portion of the follicular wall,with transepidermal elimination of connec-tive tissue elements and cellular debris

PathogenesisTheories of causation: premature follicularkeratinization; primary alteration of con-nective tissue or deposition of foreignmaterial within the superficial dermis, withsubsequent engulfment and elimination byproliferative follicular epithelium; coiled

hairs which disrupt the follicular epithe-lium

Clinical manifestationAssociation with diabetes mellitus andrenal failure; papules concentrated on hair-bearing portions of the extremities and but-tocks; chronic, recurrent, asymptomatic, ormildly pruritic, scaly papules, often follicu-locentric, with small central keratotic plugsand varying degrees of erythema; sponta-neous remission may occurr

Differential diagnosisFolliculitis; acne; pseudofolliculitis barbae;elastosis perforans serpiginosa; Kyrle dis-ease; reactive perforating collagenosis; per-forating granuloma annulare; prurigo nod-ularis; insect bite reaction

TherapyTretinoin

ReferencesChang P, Fernandez V (1993) Acquired perforat-

ing disease: report of nine cases. International Journal of Dermatology 32(12):874–876

Penicillin VK. Dermatologic indications and dosage




25–50 mg per kg PO divided into 4 doses daily for 10 days

Anthrax 250–500 mg PO for up to 60 days in bioterrorism situation

25–50 mg per kg PO divided into 4 doses daily for up to 60 days in bioterrorism situation




Erysipelas 250–500 mg PO 4 times daily for 10–14 days

25–50 mg per kg daily PO divided into 4 doses for 10–14 days

Erysipeloid 250–500 mg PO 4 times daily for 10 days




Scarlet fever 500 mg PO for 10 days 25–50 mg per kg daily divided into 4 doses PO for 10 days


452 Perfume phototoxicity

Perfume phototoxicity


Periadenitis mucosa necrotica recurrens

� Aphthous stomatitis

Perianal dermatitis


Periarteritis nodosa

� Polyarteritis nodosa

Perifolliculitis capitis abscedens et suffodiens


Perineural fibroblastoma

� Neurilemmoma

Perineural myxoma

� Neurothekeoma

Perioral dermatitis

Synonym(s)Rosacea-like dermatitis; periorificial der-matitis; light-sensitive seborrheid; chronicpapulopustular facial dermatitis; granulo-matous perioral dermatitis; steroid rosacea

DefinitionChronic facial dermatitis, occurring mostlyin young women, characterized by smallred papules and pustules around themouth, nose, and eyes

PathogenesisAssociated with topical corticosteroid useon the face

Clinical manifestationGrouped follicular papules, papulovesicles,and papulopustules on an erythematousbase, may evolve into plaques; locatedmainly in perioral area, but also in nasola-bial fold and lateral portions of the lowereyelids

Differential diagnosisHaber syndrome; acne vulgaris; rosacea;seborrheic dermatitis; lupus erythemato-sus; tinea faciei; contact dermatitis

TherapyDiscontinuance of all topical steroid use tothe face�; tetracycline; doxycycline; minoc-ycline; erythromycin

ReferencesKuflik JH, Janniger CK, Piela Z (2001) Perioral

dermatitis: an acneiform eruption. Cutis 67(1):21–22

Periorificial dermatitis



Persistent light reactivity 453

P

Perleche

� Candidiasis

Permethrin

Trade name(s)Elimite; Nix

Generic availableNo

Drug classAnti-parasitic agent

Mechanism of actionNeural transmission blockade

Dosage form5% cream; 1% cream rinse


Common side effectsCutaneous: pruritus, redness, scalp swelling




ReferencesWendel K, Rompalo A (2002) Scabies and pedicu-

losis pubis: an update of treatment regimens and general review. Clinical Infectious Diseases 35(Suppl 2):S146–151

Pernio

� Chilblains

Perniosis

� Chilblains

Persistent light reaction


Persistent light reactivity


Permethrin. Dermatologic indications and dosage

Dosage Adult dosage Child dosage

Pediculosis capitis and pubis

Apply 1% cream rinse for 10 minutes; remove nits with comb provided with medication

Apply 1% cream rinse for 10 minutes; remove nits with comb provided with medication

Scabies Apply 5% cream over whole skin surface below the neck; repeat in 7 days

Apply 5% cream over whole skin surface below the neck; repeat in 7 days


454 Peutz-Jeghers syndrome

Peutz-Jeghers syndrome

Synonym(s)None

DefinitionHereditary syndrome characterized by mul-tiple gastrointestinal polyps and mucocuta-neous pigmentation

PathogenesisAutosomal dominant trait; germline muta-tion of STK11 (serine threonine kinase 11)gene; protein likely regulated by phosphor-ylation by cAMP-dependent proteinkinase A

Clinical manifestationDozens to thousands of hamartomatouspolyps in stomach and intestines, primarilyin the small intestine; macular hyperpig-mentation on inner lining of the mouth,gums, lips, around the mouth, around theeyes, fingers or toes, and genitalia; pigmen-tation varying in color from bluish black todark brown to blue; lesions may fade overtime

Differential diagnosisFamilial adenomatous polyposis; Cowdendisease; juvenile polyposis; Ruvalcaba-Myhre-Smith; Turcot syndrome; Carney’ssyndrome; Cronkhite-Canada syndrome

TherapyNo treatment for mucocutaneous pigmen-tation; repeated gastrointestinal endo-scopic examinations with surgical removalof polyps suspicious for malignancy

ReferencesMcGarrity TJ, Kulin HE, Zaino RJ (2000) Peutz-

Jeghers syndrome. American Journal of Gas-troenterology 95(3):596–604

Peyronie disease



Synonym(s)Peyronie disease; plastic induration of thepenis; penile fibromatosis; fibrous sclerosisof the penis; Van Buren’s disease

DefinitionSyndrome characterized by penile deform-ity and painful erection secondary to fibro-sis of the tunica albuginea

PathogenesisMicrotraumatic events during intercoursemay be part of the cause; associated withDupeytron’s contracture in some patients;possibly associated with erectile dysfunc-tion, diabetes mellitus, and hypertension,with partial erections leading to bucklingduring intercourse

Clinical manifestationPenile pain and curvature during erection;fibrotic plaque over the midline of thepenile shaft, either ventrally or dorsally;stabilization of signs and symptoms overtime in many patients

Differential diagnosisScleroderma; lichen sclerosus; congenitalpenile curvature; penile fracture; peniledorsal vein thrombosis; leukemic infiltrateof the penis; syphilis; lymphogranulomavenereum

TherapySurgical correction�

ReferencesKadioglu A, Tefekli A, Erol B, Oktar T, Tunc M,

Tellaloglu S (2002) A retrospective review of 307 men with Peyronie's disease. Journal of Urology 168(3):1075–1079

Pfeifer-Weber-Christian syndrome

� Weber-Christian disease


Phytosterolemia 455

P

Pfeiffer’s syndrome

� Acrocephalosyndactyly

Phaeohyphomycosis


Photochemotherapy

� Methoxsalen

Photodermatitis pigmentaria


Photosensitive eczema


Photosensitivity dermatitis


Phototoxic dermatitis


Phthiriasis

� Pediculosis

Phycomycosis

� Mucormycosis

Phytosterolemia

Synonym(s)Sitosterolemia; pseudohomozygous famil-ial hypercholesterolemia

DefinitionInherited plant sterol storage disease, char-acterized by tendon and tuberous xantho-mas and a strong tendency to develop pre-mature coronary atherosclerosis

PathogenesisAutosomal recessive disorder; mutations ineither of the genes for two proteins (ABCG5or ABCG8) that preferentially pump plantsterols out of intestinal cells into the gutlumen and out of liver cells into the bileducts, thereby decreasing sterol absorp-tion; hyperabsorption of sitosterol from thegastrointestinal tract; decreased hepaticsecretion of sitosterol with subsequentdecreased elimination; dysfunctional cho-lesterol synthesis

Clinical manifestationXanthomas at any age, even in childhood;xanthelasma and corneal arcus; signs ofpremature coronary vascular disease, suchas congestive heart failure; decreased rangeof motion and/or redness, swelling, andwarmth of joints due to arthritis; splenome-galy


456 Pian

Differential diagnosisFamilial hypercholesterolemia; pseudo-homozygous familial hypercholestero-lemia; cerebrotendinous xanthomatosis;lipid storage disorders

TherapyDiet low in plant sterols�; cholestyramine:3–4 g/d PO tid; ileal bypass surgery

ReferencesLing WH, Jones PJ (1995) Dietary phytosterols: a

review of metabolism, benefits and side effects. Life Sciences 57(3):195–206

Pian

� Yaws

Pick disease


Pick’s disease


Picker’s acne

� Acne excoriée

Piebaldism

Synonym(s)Partial albinism; familial white spotting

DefinitionFamilial disorder characterized by congeni-tal white forelock and multiple symmetri-cal hypopigmented or depigmented mac-ules and patches

PathogenesisAutosomal trait; mutations of the KITproto-oncogene

Clinical manifestationWhite forelock, with both hair and skin inthe central frontal scalp often in triangularshape; permanently white from birth orwhen hair color first becomes apparent;may affect eyebrow and eyelash hair; sym-metrical, irregular, hypopigmented mac-ules and patches on face, trunk, andextremities; depigmented skin, sometimesshowing narrow border of hyperpigmenta-tion or island of pigmentation

Differential diagnosisVitiligo; albinism; nevus depigmentosus;hypomelanosis of Ito; Waardenburg’s syn-drome; chemical leukoderma; onchocercia-sis; preus syndrome; pinta; Vogt-Koyanagi-Harada syndrome; leprosy; tinea versi-color; pityriasis alba


ReferencesLe Poole C, Boissy RE (1997) Vitiligo. Seminars in

Cutaneous Medicine & Surgery 16(1):3–14

Piedra

Synonym(s)Black piedra, white piedra, trichosporosis,tinea nodosa; trichomycosis nodularis

DefinitionSuperficial fungal infection of the hairshafts, resulting in the formation of smallnodules


Pigmented contact dermatitis 457

P

PathogenesisTwo pathogenic fungal organisms: Piedraiahortae causing black piedra; Trichosporonbeigelii causing white piedra

Clinical manifestationBlack piedra: firmly adherent, black, firm,oval or elongated papules, composed of amass of fungus cells; scalp most commonsite of involvement, but also seen in thebeard and pubic areasWhite piedra: soft, white or light-brownpapules loosely adherent to or within thehair shaft; scalp most common site ofinvolvement, but also seen in the beard andpubic areas; increased carriage rate in HIV-positive patients; may be sexually transmit-ted

Differential diagnosisPediculosis; tinea capitis; tinea corporis;trichomycosis axillaris

TherapyShaving or cutting the affected hair�

Black piedra: terbinafineWhite piedra: topical azole antifungalagents; ciclopirox cream; itraconazole forrecalcitrant disease

ReferencesDrake L, Dinehart S, Farmer E, Goltz RW, et al.

(1996) Guidelines for care for superficial my-cotic infections of the skin: piedra. Journal of the American Academy of Dermatology 34(1):122–124

Piezogenic papule

Synonym(s)Piezogenic pedal papule; painful piezo-genic papule

DefinitionPapules of the feet resulting from hernia-tion of fat through the dermis

PathogenesisResults from fat herniation into the dermis

Clinical manifestationAsymptomatic or painful, flesh-coloredpapules over medial, posterior, and lateralaspects of the heels, usually occurring bilat-erally; more common in overweight peo-ple, those with flat feet, with Ehlers-Danlossyndrome, or those who spend significanttime on their feet

Differential diagnosisWart; benign adnexal tumor; foreign bodygranuloma

TherapyNo effective curative therapy; heel cup inshoe to minimize herniation

ReferencesPontious J, Lasday S, Mele R (1990) Piezogenic

pedal papules extending into the arch. Case re-port and discussion. Journal of the American Podiatric Medical Association 80(8):444–445

Piezogenic pedal papule

� Piezogenic papule

Pigmented contact dermatitis


Piezogenic papule. Flesh-colored papules on the heel of the foot


458 Pigmented cosmetic dermatitis

Pigmented cosmetic dermatitis


Pigmented hairy epidermal nevus


Pigmented pretibial patches


Pigmented purpuric dermatitis


Pigmented purpuric eruption


Pigmented purpuric lichenoid dermatosis of Gougerot and Blum


Pigmented reticular dermatosis of the flexures


Pilar cyst

Synonym(s)Trichilemmal cyst; scalp cyst; wen;keratinous cyst

DefinitionKeratin-producing cyst, derived from thehair follicle outer root sheath, usuallyappearing on the scalp

PathogenesisDerived from outer root sheath of hair folli-cle; may begin as budding from externalroot sheath as a genetically determinedstructural aberration

Clinical manifestationSmooth, firm, subcutaneous nodule, usu-ally on the scalp, without punctum, con-taining keratinous material

Differential diagnosisEpidermoid cyst; pilomatricoma; dermoidcyst; lipoma; organized hematoma


ReferencesBulengo-Ransby SM, Johnson C, Metcalf JS (1995)

Enlarging scalp nodule. Proliferating trichi-lemmal cyst (PTC). Archives of Dermatology 131(6):721,724


Pili trianguli canaculi 459

P

Pilar tumor

Synonym(s)Proliferating pilar tumor; proliferating tri-chilemmal cyst

DefinitionNeoplasm derived from follicular outer rootsheath, characterized by a large exophyticnodule, usually on the scalp

PathogenesisArises as neoplastic transformation of pilarcyst; may be associated with trauma, irrita-tion, or inflammation

Clinical manifestationAsymptomatic, large, flesh-colored nodule;sometimes having inflammation, ulcera-tion, bleeding, and/or yellowish discharge;occasional malignant degeneration

Differential diagnosisPilar cyst; lipoma; cylindroma; squamouscell carcinoma; cutaneous metastasis

TherapyComplete surgical excision�

ReferencesMathis ED, Honningford JB, Rodriguez HE, Wind

KP, Connolly MM, Podbielski FJ (2001) Malig-nant proliferating trichilemmal tumor. Ameri-can Journal of Clinical Oncology 24(4):351–353

Pili annulati (ringed hairs)

DefinitionHair with alternating light and dark bands

ReferencesMoffitt DL, Lear JT, de Berker DA. Peachey RD

(1998) Pili annulati coincident with alopecia ar-eata. Pediatric Dermatology 15(4):271–273

Pili bifurcati

DefinitionHairs arising from single papilla and thendividing into separate shafts

ReferencesCamacho FM, Happle R, Tosti A, Whiting D

(2000) The different faces of pili bifurcati. A re-view. European Journal of Dermatology 10(5):337–340

Pili incarnati


Pili torti

DefinitionHair shaft that is flattened and twisted onits own axis, usually through 180º angle

ReferencesRogers M (1995) Hair shaft abnormalities: Part I.

Australasian Journal of Dermatology 36(4):179–184

Pili trianguli canaculi

DefinitionUncombable hair syndrome characterizedby hair shafts that are triangular in cross-section

ReferencesHicks J, Metry DW, Barrish J, Levy M (2001) Un-

combable hair (cheveux incoiffables, pili trian-guli et canaliculi) syndrome: brief review and role of scanning electron microscopy in diag-nosis. Ultrastructural Pathology 25(2):99–103


460 Pili trianguli et canaliculi

Pili trianguli et canaliculi


Pilomatricoma

Synonym(s)Pilomatrixoma; calcifying epithelioma ofMalherbe; trichomatrioma; benign calcify-ing epithelioma of Malherbe

DefinitionBenign tumor of skin appendage, with dif-ferentiation toward hair matrix cells

PathogenesisMay involve faulty suppression of apopto-sis, with beta-catenin/LEF dysregulation

Clinical manifestationFlesh-colored, firm nodule, often in thehead and neck area; usually asymptomatic,but sometimes painful during episodes ofinflammation

Differential diagnosisEpidermoid cyst; basal cell carcinoma;trichilemmoma; trichoepithelioma; calcino-sis cutis; cutaneous tuberculosis; granu-loma annulare; sarcoidosis; cutaneousmetastasis; Merkel cell carcinoma; osteomacutis; dermatofibrosarcoma protuberans


ReferencesSassmannshausen J, Chaffins M (2001) Pilomatrix

carcinoma: a report of a case arising from a previously excised pilomatrixoma and a review of the literature. Journal of the American Acad-emy of Dermatology 44(2 Suppl):358–361

Pilomatrixoma

� Pilomatricoma

Pincer nail

DefinitionExcessive transverse curvature of the nailplate, often of the great toe, with groovinginto the lateral and medial nail fold

ReferencesBaran R, Haneke E, Richert B (2001) Pincer nails:

definition and surgical treatment. Dermatolog-ic Surgery 27(3):261–266

Pink disease

� Acrodynia

Pinkus tumor

� Fibroepithelioma of Pinkus

Pinta

Synonym(s)Azul; carate; endemic treponematosis; malde pinto

DefinitionBacterial infection of the skin caused by atreponemal pathogen, characterized by


Pitted keratolysis 461

P

papules and plaques in the early stage anddyschromic patches in the late stage

PathogenesisTreponema carateum is causative agent,separate species from Treponema palli-dum, the cause of syphilis; unclear mode oftransmission; possibly transmitted by skin-to-skin contact

Clinical manifestationPapule that slowly enlarges to become pru-ritic plaque; dorsum of foot and legs mostcommon sites; regional lymphadenopathy;lesions become pigmented with age; some-times copper to gray to slate; late lesions areachromic or hyperpigmented

Differential diagnosisSyphilis; yaws; leprosy; tinea corporis; tineaversicolor; vitiligo; post-inflammatoryhypopigmentation; pityriasis alba

TherapyPenicillin G�; therapy for penicillin-aller-gic patients: tetracycline; erythromycin

ReferencesParish JL (2000) Treponemal infections in the

pediatric population. Clinics in Dermatology 18(6):687–700

Pitted keratolysis

Synonym(s)Keratoma plantarum sulcatum; keratolysisplantaris sulcatum; ringed keratolysis

DefinitionBacterial infection characterized by crateri-form pitting primarily affecting the pres-sure-bearing aspects of the plantar surfaceof the feet

PathogenesisInfection with Micrococcus sedentarius,Dermatophilus congolensis, or species of

Corynebacterium or Actinomyces; underappropriate conditions (i.e., prolongedocclusion, hyperhidrosis, increased skinsurface pH), bacterial proliferate and pro-duce proteinases that destroy stratum cor-neum, creating pits; malodor secondary toproduction of sulfur-compound by-prod-ucts

Clinical manifestationPits in stratum corneum, with some conflu-ence, irregular erosions, or sulci, most oftenon plantar aspects of feet; usually asympto-matic, but may have malodor, hyperhidro-sis, sliminess, and occasionally soreness oritching

Differential diagnosisPlantar warts; tinea pedis; essential hyper-hidrosis; basal cell nevus syndrome; kera-tolysis exfoliativa; punctate keratoderma;arsenical keratoses

TherapyErythromycin, topical; clindamycin, topi-cal; erythromycin, oral; limited use ofocclusive footwear; reduced foot frictionwith properly fitting shoes; absorbent cot-ton socks, changed frequently

ReferencesOmura EF, Rye B (1994) Dermatologic disorders

of the foot. Clinics in Sports Medicine 13(4):825–841

Pitted keratolysis. Pits on the plantar aspect of the foot


462 Pityriasis alba

Pityriasis alba

Synonym(s)Pityriasis simplex; pityriasis sicca faciei

DefinitionDisorder characterized by asymptomatic,scaly, variably hypopigmented plaques,mostly occurring in children

PathogenesisAssociated with atopic diathesis; may repre-sent post-inflammatory change

Clinical manifestationSolitary or multiple, rounded, oval, or irreg-ular plaques that are red, pink, or skincolored, with pityriasiform scale, mostoften on the face, neck, and lateral arms;occurs mainly in children

Differential diagnosisTinea corporis; tinea versicolor; sarcoido-sis; vitiligo; psoriasis; leprosy; mycosis fun-goides; seborrheic dermatitis; nummulareczema

TherapyCorticosteroids, topical, low potency�;emollients

ReferencesGalan EB, Janniger CK (1998) Pityriasis alba.

Cutis 61(1):11–13

Pityriasis corporis

� Seborrheic dermatitis

Pityriasis lichenoides

Synonym(s)Mucha-Habermann disease; guttate parap-soriasis; pityriasis lichenoides chronica;pityriasis lichenoides et varioliformis acuta

DefinitionDisease spectrum ranging from an acutepapulovesicular eruption to a chronic erup-tion consisting of small, scaly, red papules

PathogenesisUnclear whether two distinct diseases orvariants of same process; acute disease(Mucha-Habermann disease) may behypersensitivity reaction to infectious agentor some other environmental insult

Clinical manifestationAcute variant (Mucha-Habermann dis-ease): abrupt appearance of multiple pru-ritic papules on the trunk, buttocks, andproximal extremities, evolving to vesicleswhich rupture and produce hemorrhagiccrusts; lesions heal with postinflammatoryleukoderma or hyperpigmentation; mayhave lesions identical to those of chronicvariantChronic variant (pityriasis lichenoideschronica): at the subacute end of spectrum,may develop over days; distributed over thetrunk, buttocks, and proximal extremities;small, erythematous-to-reddish brownpapules, with fine scale; often polymor-phic, with lesions at different stages of evo-lution

Differential diagnosisAcute variant: varicella; vasculitis; scabies;dermatitis herpetiformis; external trauma;insect bite reactionChronic variant: psoriasis; small plaqueparapsoriasis; mycosis fungoides; tinea cor-poris; lupus erythematosus; pityriasisrosea; syphilis; viral exanthem

TherapyAcute variant: methotrexate; tetracycline;erythromycin; photochemotherapyChronic variant: phototherapy; photochem-otherapy; corticosteroids, topical, highpotency

ReferencesPatel DG, Kihiczak G, Schwartz RA, Janniger CK

Lambert WC (2000) Pityriasis lichenoides. Cutis 65(1):17–20,23


Pityriasis rubra pilaris 463

P

Pityriasis lichenoides chronica


Pityriasis lichenoides et varioliformis acuta


Pityriasis oleosa


Pityriasis pilaris


Pityriasis rosea

Synonym(s)None

DefinitionSelf-limited eruption consisting of multi-ple, oval, scaling papules often preceded bya single larger plaque known as “heraldpatch”

PathogenesisMay be viral exanthem, although no virusconsistently isolated

Clinical manifestationHerald patch: single (or few) annular, scalyplaque(s), on neck or trunk; several days

after herald patch, onset of multiple,salmon-colored, scaly papules; long axes ofthe lesions oriented in parallel fashionalong cleavage lines; occurs on the trunk,abdomen, back, and the proximal upperextremities; eruption clears in 6–12 weeks,with only rare recurrences

Differential diagnosisSyphilis; pityriasis lichenoides; tinea cor-poris; mycosis fungoides; lupus erythema-tosus; drug eruption; viral exanthem; num-mular eczema; seborrheic keratosis

TherapyErythromycin; UVB phototherapy

ReferencesNelson JS, Stone MS (2000) Update on selected vi-

ral exanthems. Current Opinion in Pediatrics 12(4):359–364

Pityriasis rubra pilaris

Synonym(s)None

DefinitionChronic disorder characterized by reddish-orange scaling plaques, palmoplantar kera-toderma, and keratotic follicular papules

Pityriasis rubra pilaris. Marked scale and erythema of the palms


464 Pityriasis sicca

PathogenesisUnknown

Clinical manifestationOrange-red or salmon-colored scalingplaques with sharp borders, which mayexpand to become whole body erythro-derma, with islands of sparing; follicularhyperkeratosis on the dorsal aspects of theproximal phalanges, elbows, and wrists;palmoplantar hyperkeratosis; nails withdistal yellow-brown discoloration, subun-gual hyperkeratosis, longitudinal ridging,nail plate thickening, and splinter hemor-rhagesSubtypes:Type I: most common form; acute onset oferythroderma with islands of sparing, pal-moplantar keratoderma, and follicularhyperkeratosis; 80% of patients have remis-sion in about 3 yearsType II: ichthyosiform lesions; areas ofeczematous change; alopecia; long durationof diseaseType III: very similar to type I, but onsetwithin the first 2 years of lifeType IV: occurs in prepubertal children;sharply demarcated areas of follicularhyperkeratosis and erythema of the kneesand elbows, without progressionType V: most cases of familial diseasebelong to this group; early onset andchronic course; prominent follicular hyper-keratosis; scleroderma-like changes on thepalms and soles; infrequent erythemaType VI: HIV-associated; nodulocystic andpustular acneiform lesions; resistant tostandard treatments but sometimesresponds to antiretroviral therapies

Differential diagnosisPsoriasis; erythroderma variabilis; othercauses of exfoliative erythroderma, includ-ing T-cell lymphoma, drug eruption, atopicdermatitis, pemphigus foliaceus, and sebor-rheic dermatitis

TherapyMethotrexate; cyclosporine; acitretin; thio-guanine

ReferencesAlbert MR, Mackool BT (1999) Pityriasis rubra

pilaris. International Journal of Dermatology 38(1):1–11

Pityriasis sicca


Pityriasis sicca faciei

� Pityriasis alba

Pityriasis simplex

� Pityriasis alba

Pityriasis simplex capitis


Pityriasis versicolor


Pityriasis vulgaris


Pityrosporom folliculitis

� Folliculitis


Plastic induration of the penis 465

P

Planar xanthoma

� Xanthoma

Plane xanthoma

� Xanthoma

Plantar fibromatosis

Synonym(s)Ledderhose disease

DefinitionHeterogeneous group of conditions inplantar location with histologic features ofmature collagen and fibroblasts with nomalignant cytologic features

PathogenesisAssociated with repeated trauma, long-termalcohol consumption, chronic liver disease,diabetes mellitus; may have other fibrosingconditions such as Dupeytron’s contrac-ture, knuckle pads, or Peyronie disease

Clinical manifestationOne or more small, asymptomatic, slowlyprogressive, round or flattened, hard nod-ules generally located on the medial side ofthe sole, often bilaterally symmetrical

Differential diagnosisDesmoid tumor; keloid/hypertrophic scar;granuloma annulare; calcinosis cutis;mucocoele; dermatofibrosarcoma protuber-ans; neurofibroma; neuroid nevus;melanoma; osteoma; gout

TherapyFasciotomy and excision of the fibrous tis-sue for symptomatic lesions

ReferencesGodette GA, O'Sullivan M, Menelaus MB (1997)

Plantar fibromatosis of the heel in children: a report of 14 cases. Journal of Pediatric Ortho-pedics 17(1):16–17

Plantar wart

� Wart

Plaque-like dermal fibromatosis

� Dermatomyofibroma

Plasma cell balanitis

� Zoon balanitis

Plasma cell balanitis of Zoon

� Zoon balanitis

Plasma cell mucositis

� Zoon balanitis

Plastic induration of the penis



466 Plumber's itch

Plumber's itch

� Cutaneous larva migrans

Pluriorificial keratosis of Olmsted

� Olmsted syndrome

Podofilox

Trade name(s)Condylox

Generic availableNo

Drug classPodophyllum resin (podophyllin)

Mechanism of actionInhibits microtubular function by combin-ing with a component of microtubules

Dosage form0.5% solution, gel


Common side effectsCutaneous: burning sensation, irritant der-matitis




ReferencesBeutner KR (1996) Podophyllotoxin in the treat-

ment of genital warts. Current Problems in Dermatology 24:227–232

Podophyllin

� Podofilox

POEMS syndrome

Synonym(s)Crow-Fukase syndrome; Takatsuki syn-drome

DefinitionMultisystem disease consisting of polyneu-ropathy, organomegaly, endocrinologic dis-orders, monoclonal gammopathy, and vari-ous skin abnormalities

PathogenesisPlasma cell disorder central to other find-ings

Podofilox. Dermatologic indications and dosage


Bowenoid papulosis Apply twice daily for 3 consecutive days weekly, up to 4 weeks

Not indicated

Genital wart Apply twice daily for 3 consecutive days weekly, up to 4 weeks

Not indicated


Poikiloderma of Kindler 467

P

Clinical manifestationSkin manifestations: diffuse hyperpigmen-tation; lower extremity edema; hypertricho-sis, usually most pronounced over the face,limbs, and chest; sclerodermoid changes;angiomas; whitening of the proximal nails;hepatomegaly; splenomegaly; lymphaden-opathy; abnormal estrogen levels withgynecomastia; hypothyroidism; hyperprol-actinemia; hypoparathyroidismNeurologic manifestations: progressivebilateral symmetric disturbances involvingboth motor and sensory nerves; begins dis-tally and has progressive proximal spread;osteosclerotic myeloma or monoclonalgammopathy

Differential diagnosisScleroderma; Raynaud disease; multiplemyeloma; Addison’s disease

TherapyTreatment of underlying plasma cell disor-der with corticosteroids and chemotherapyand/or radiation therapy; surgical excisionof isolated plasmacytoma

ReferencesKoike H, Sobue G (2000) Crow-Fukase syndrome.

Neuropathology. 20 Suppl:S69–72

Poikiloderma atrophicans vasculare

Synonym(s)None

DefinitionTerm used to describe plaques with ciga-rette paper-like atrophy, telangiectasia, andmottled hyperpigmentation


ReferencesHoward MS, Smoller BR (2000) Mycosis fun-

goides: classic disease and variant presenta-tions. Seminars in Cutaneous Medicine & Surgery 19(2):91–99

Poikiloderma congenitale

� Rothmund-Thomson syndrome

Poikiloderma of Civatte

Synonym(s)Berkshire neck

DefinitionErythema and mottled pigmentation seenon the sides of the neck, related to chronicsun exposure

PathogenesisAssociated with chronic sun exposure infair-skinned individuals

Clinical manifestationReddish-brown reticulate pigmentationwith atrophy and telangiectasia, usually insymmetrical plaques on sides of the neck

Differential diagnosisPoikiloderma atrophicans vasculare; Roth-mund-Thomson syndrome; Bloom syn-drome; lupus erythematosus; dermatomy-ositis; berloque dermatitis; Riehl’s melano-sis

TherapyIntense pulsed-light (IPL) source; flash-lamp-pumped pulse dye laser (FPDL,585 nm); potassium-titanyl-phosphate(KTP) laser

ReferencesRoss BS, Levine VJ, Ashinoff R (1997) Laser treat-

ment of acquired vascular lesions. Dermatolog-ic Clinics 15(3):385–396

Poikiloderma of Kindler

� Kindler syndrome


468 Poikiloderma vasculare atrophicans

Poikiloderma vasculare atrophicans


Polyarteritis nodosa

Synonym(s)Periarteritis nodosa

DefinitionSystemic vasculitis characterized by necro-tizing inflammatory lesions affecting pre-dominately medium and small musculararteries

PathogenesisMay be immune complex-mediated processin hepatitis B-associated disease

Clinical manifestationConstitutional signs and symptoms: fever;weight loss; myalgias; abdominal painSkin findings: palpable purpura; cutaneousinfarctions with ulceration, discontinuouslivedo reticularis (retiform purpura);ischemic changes of the distal digits; subcu-taneous nodules; purely cutaneous involve-ment sometimes occurs; may have myal-gias, arthralgias, and peripheral neuropathySystemic disease: mesenteric thrombosisand ischemia; renal vascular nephropathy;sensory and motor neuropathies; monone-uritis multiplex; coronary arteritis; tachy-cardia; retinal vasculitis

Differential diagnosisMicroscopic polyangiitis; septicemia, infec-tive endocarditis, malignancy; atheroscle-rosis; rheumatoid arthritis; Sjögren syn-drome; cryoglobulinemia; lupus erythema-tosus

TherapyPrednisone�; cyclophosphamide; cyclo-sporine

ReferencesGuillevin L (1999) Treatment of classic polyarteri-

tis nodosa in 1999. Nephrology Dialysis Trans-plantation 14(9):2077–2079

Polychondropathy


Polykeratosis congenita

� Pachyonychia congenital

Polymorphic eruption of pregnancy

� Pruritic urticarial papules and plaques of pregnancy

Polymorphic light eruption

� Polymorphous light eruption

Polymorphic prurigo syndrome


Polymorphic reticulosis



Polyostotic fibrous dysplasia 469

P


Synonym(s)Polymorphic light eruption

DefinitionPhotodermatosis characterized by recur-rent, abnormal, delayed reactions to sun-light, ranging from erythematous papules,papulovesicles, and plaques to erythemamultiforme-like lesions

PathogenesisUltraviolet A (UVA) light causative in mostcases; mechanism of inflammatoryresponse unclear; immunologic factorsprobably important

Clinical manifestationOften seen at onset of a vacation in a sunnyplace or at high altitude; sun-exposed skin,especially that normally covered in winter,most commonly affected; improves as thesummer progresses; eruption appearswithin hours to days of exposure and sub-sides over 1–7 days without scarring; pru-ritic papules (most common), plaques,papulovesicles, and erythema multiforme-like lesions, often combined in the samepatient; small papular lesions sometimescoalesce to form eczematous plaque; auto-sensitization sometimes leads to a general-

ized involvement; cheilitis occurs mainly inNative American children with a combinedpolymorphous light and atopic dermatitis-like syndrome (actinic prurigo)

Differential diagnosisSolar urticaria; lupus erythematosus; eryth-ropoietic protoporphyria; actinic dermati-tis; hydroa vacciniforme; drug-inducedphotosensitivity

TherapyProphylactic broadband UVB photother-apy before the onset of the sunny season;prophylactic photochemotherapy (PUVA)before the onset of the sunny season; pro-phylactic narrowband UVB phototherapybefore the onset of the sunny season;hydroxychloroquine; thalidomide; beta car-otene: 120–300 mg PO per day; niacina-mide: 1000 mg PO 3 times daily for 2 weeks;corticosteroids, topical, high potency; pred-nisone for severe acute flares

ReferencesNaleway AL. Polymorphous light eruption. Inter-

national Journal of Dermatology 41(7):377–383

Polymorphous prurigo syndrome


Polyostotic dysplasia

� McCune-Albright Syndrome

Polyostotic fibrous dysplasia


Polymorphous light eruption. Flesh-colored and eroded papules on the face and lips


470 Polythelia

Polythelia

� Supernumerary nipple

Pomade acne

Synonym(s)None

DefinitionForm of acne occurring in those usingheavy pomades on the hair

PathogenesisComedones caused by heavy oils inpomades, which plug sebaceous follicles;other chemicals in pomades may be irritat-ing to skin

Clinical manifestationMultiple comedones with few inflamma-tory papules on scalp, forehead, and tem-ples

Differential diagnosisMilia; nevus comedonicus; Favre-Racou-chot disease; radiation acne; chloracne; flatwarts; appendageal tumors (syringoma,etc.); sebaceous gland hyperplasia

TherapyTretinoin�; avoidance of comedogenicagents on scalp

ReferencesLaude TA (1995) Approach to dermatologic disor-

ders in black children. Seminars in Dermatolo-gy 14(1):15–20

Pompholyx


Ponytail band alopecia


Popsicle panniculitis

� Cold panniculitis

Porokeratosis

Synonym(s)Porokeratosis of Mibelli; disseminatedsuperficial actinic porokeratosis; DSAP;porokeratosis palmaris et plantaris dissem-inata; linear porokeratosis; punctate poro-keratosis; hyperkeratosis eccentrica;hyperkeratosis figurata centrifuga atrophi-cans

DefinitionKeratotic lesion characterized by periph-eral spread, a thin thready border, and anatrophic center

PathogenesisClonal hyperproliferation of atypical kerati-nocytes leading to the formation of the cor-noid lamella, which forms the boundary

Porokeratosis. Plaque with thready border of scale


Poroma 471

P

between abnormal and normal keratinoc-ytes; loss of heterozygosity may be mecha-nism for linear porokeratosis; genetic fac-tors in disseminated superficial actinicporokeratosis

Clinical manifestationPorokeratosis of Mibelli: slowly expanding,irregularly shaped plaque with a raised,thready border; lesion slightly hypopig-mented or hyperpigmented, minimallyscaly, hairless, slightly atrophic, and anhid-roticDisseminated superficial actinic porokera-tosis: multiple, small, indistinct, lightbrown papules with a threadlike border, onthe extensor surface of upper and lowerextremitiesLinear porokeratosis: grouped, linear, annu-lar papules and plaques with a raisedperipheral ridge on an extremity, the trunk,and/or the head and neck area, often in adermatomal distribution

Differential diagnosisActinic keratosis; squamous cell carci-noma; granuloma annulare; superficialbasal cell carcinoma; annular lichen pla-nus; elastosis perforans serpiginosa; flatwarts

TherapyFluorouracil cream; imiquimod 5% creamapplied 3 times weekly for 4–8 weeks;destruction by liquid nitrogen cryotherapyor by electrodesiccation and curettage; cal-cipotriene; isotretinoin; dermabrasion

ReferencesSehgal VN, Jain S, Singh N (1996) Porokeratosis.

Journal of Dermatology 23(8):517–525

Porokeratosis of Mibelli

� Porokeratosis

Porokeratosis palmaris et plantaris disseminata

� Porokeratosis

Poroma

Synonym(s)Eccrine poroma; apocrine poroma;juxtaepidermal poroma; hidroacanthomasimplex; dermal duct tumor

DefinitionAdnexal neoplasm composed of benign epi-thelial cells that show tubular (usually duc-tal) differentiation of either eccrine or apo-crine lineage

PathogenesisUnknown

Clinical manifestationAsymptomatic, solitary, slow-growing, orstable papule or nodule; exophytic lesionssometimes have surface erosion or ulcera-tion; may appear as if erupting through acollarette; eccrine variant almost always onthe palm or sole

Differential diagnosisAcrospiroma; pyogenic granuloma;melanoma; hidradenoma; wart; callus; for-eign body reaction


ReferencesKamiya H, Oyama Z, Kitajima Y (2001)

“Apocrine” poroma: review of the literature and case report. Journal of Cutaneous Patholo-gy 28(2):101–104


472 Porphyria

Porphyria

Synonym(s)None

DefinitionGroup of inherited disorders involvingabnormalities in the production of heme,resulting in abnormal accumulations ofporphyrins

ReferencesSassa S (2002) The porphyrias. Photodermatolo-

gy, Photoimmunology & Photomedicine 18(2):56–67

Porphyria cutanea tarda

Synonym(s)Hepatic porphyria

DefinitionGroup of related disorders arising fromdeficient activity of the heme-syntheticenzyme uroporphyrinogen decarboxylasein the liver, characterized by photosensitiv-ity eruption

PathogenesisReduced activity of uroporphyrinogendecarboxylase in hepatic heme synthesis,resulting in overproduction of porphyrinby-products of the heme biosynthetic path-way; photoexcited porphyrins in the skinmediate oxidative damage to biomoleculartargets, producing photosensitivity reaction

Clinical manifestationFragility in sun-exposed skin after mechan-ical trauma, leading to erosions and bullae,most commonly on dorsal hands, fore-arms, and face; healing of crusted erosionsand blisters leaves scars, milia, and dyspig-mentation; hypertrichosis, mostly overtemporal and malar facial areas; melasma-like hyperpigmentation of face; erythema-tous suffusion of central face, neck, upperchest, and shoulder; scarring alopecia;photo-onycholysis; scleroderma-likepapules on trunk and extremities

Differential diagnosisOther forms of porphyria; pseudoporphy-ria; bullous pemphigoid; epidermolysis bul-losa acquisita; bullous diabeticorum; bul-lous lupus erythematosus; polymorphouslight eruption

TherapyTherapeutic phlebotomy, 1 unit every 2–3 weeks until clinical response or untilhemoglobin falls below 10.5–11 gm�;hydroxychloroquine; chelation therapy withdesferrioxamine

ReferencesSarkany RP (2001) The management of porphyria

cutanea tarda. Clinical & Experimental Derma-tology 26(3):225–232

Porphyria erythropoietica

� Erythropoietic porphyria

Porphyria cutanea tarda. Numerous eroded papules and scars over the dorsal aspects of the hands


Postinflammatory hyperpigmentation 473

P

Porphyria variegata


Port-wine mark

� Nevus flammeus

Port wine stain

� Nevus flammeus

Posterior lingual papillary atrophy

� Median rhomboid glossitis

Postinfectious cockade purpura of early childhood


Postinflammatory anetoderma of Jadassohn and Pellizzari

� Anetoderma

Postinflammatory hypermelanosis

� Postinflammatory hyperpigmenta-tion


Synonym(s)Postinflammatory hypermelanosis; mela-notic hyperpigmentation

DefinitionSequela of inflammatory skin disorders andtherapeutic interventions, characterized bymacular hyperpigmentation

PathogenesisEpidermal hypermelanosis: inflammatoryproducts stimulate epidermal melanocytesto increase melanin synthesis, with subse-quent increased transfer of pigment to sur-rounding keratinocytesDermal melanosis: inflammation disruptsbasal cell layer, causing melanin pigment tofall into dermis, with subsequent trappingby macrophages (pigmentary incontinence)

Clinical manifestationIrregular, light brown-to-black maculesand/or patches at sites of prior inflamma-tion

Differential diagnosisTinea versicolor; acanthosis nigricans;lichen planus; lupus erythematosus; nevoidhypermelanosis; melasma; amyloidosis;ashy dermatosis

TherapyHydroquinone; tretinoin; azelaic acid


474 Prader-Willi syndrome

ReferencesPandya AG, Guevara IL (2000) Disorders of hy-

perpigmentation. Dermatologic Clinics 18(1):91–98

Prader-Willi syndrome

Synonym(s)None

DefinitionDevelopmental syndrome consisting ofmental retardation, abnormal behavior, andhypopigmentation

PathogenesisChromosomal and molecular changes ofthe proximal region of chromosome 5

Clinical manifestationNeonatal hypotonia; hyperphagia and obes-ity; short stature; developmental delay;behavioral abnormalities; skin pigmentdilution of the skin and eyes

Differential diagnosisAngelman syndrome; oculocutaneous albi-nism

TherapySun protection

ReferencesKhan NL, Wood NW (1999) Prader-Willi and An-

gelman syndromes: update on genetic mecha-nisms and diagnostic complexities. Current Opinion in Neurology 12(2):149–154

Prednisone

Trade name(s)Deltasone; Sterapred



Mechanism of actionNuclear glucocorticoid receptor bindingand gene transcription; reduction of syn-thesis of inflammatory cells and access ofthose cells to sites of inflammation

Dosage form1 mg, 2.5 mg, 5 mg, 10 mg, 20 mg, 50 mgtablet


Common side effectsCardiovascular: hypertension, fluid reten-tionCutaneous: skin fragility and ecchymoses,skin atrophy, impaired wound healingEndocrine: Cushingoid features, hyperglyc-emiaGastrointestinal: nausea, vomiting, dyspep-sia, weight gainGenitourinary: menstrual irregularitiesInfectious: increased susceptibility to infec-tionMusculoskeletal: osteopeniaNeurologic: mood change, insomnia

Serious side effectsCardiovascular: congestive heart failureEndocrine: adrenal insufficiency uponwithdrawalGastrointestinal: peptic ulcerGenitourinary: menstrual irregularitiesInfectious: increased susceptibility to infec-tionMusculoskeletal: aseptic hip necrosisNeurologic: psychosis, pseudotumor cere-bri

Drug interactionsBarbiturates; beta agonists; COX-2 inhibi-tors; cyclosporine; digoxin; thiazide diuret-ics; glyburide/metformin; non-steroidalanti-inflammatory agents; phenytoin;rifampin; warfarin; many others


Prednisone 475

P

Prednisone. Dermatologic indications and dosage


Acne vulgaris 0.5–2 mg per kg daily PO as a single AM dose for 7–21 days

0.5–2 mg per kg daily PO as a single AM dose for 7–21 days


0.5–2 mg per kg daily PO as a single AM dose


Acute generalized exanthematous pustular dermatitis



Acute graft versus host reaction



Alopecia areata 0.5–2 mg per kg daily PO as a single AM dose


Aphthous stomatitis 0.5–2 mg per kg daily PO as a single AM dose for 7–21 days


Atopic dermatitis 0.5–2 mg per kg daily PO as a single AM dose for 7–21 days


Autoerythrocyte sensitization; erythema induratum



Behçet’s disease 0.5–2 mg per kg daily PO as a single AM dose





Bullous pemphigoid 0.5–2 mg per kg daily PO as a single AM dose


Churg-Strauss disease 0.5–2 mg per kg daily PO as a single AM dose





Contact dermatitis 0.5–2 mg per kg daily PO as a single AM dose for 7–21 days


Dyshidrotic eczema 0.5–2 mg per kg daily PO as a single AM dose for 7–21 days














Erythema gyratum repens




476 Prednisone

Erythema induratum 0.5–2 mg per kg daly PO as a single AM dose


Erythema multiforme 0.5–2 mg per kg daily PO as a single AM dose for 7–21 days


Erythema nodosum 0.5–2 mg per kg daily PO as a single AM dose for 7–21 days


Fogo selvagem 0.5–2 mg per kg daly PO as a single AM dose


Herpes gestationis 0.5–2 mg per kg daily PO as a single AM dose


Herpes zoster 0.5–2 mg per kg daily PO as a single AM dose for 7–14 days









2-4 mg per kg daily PO for 6-30 weeks

2-4 mg per kg daily PO for 6-30 weeks

Kerion 0.5–2 mg per kg daily PO as a single AM dose for 7–14 days


Kimura’s syndrome 0.5–2 mg per kg daily PO as a single AM dose


Leprosy reactional state



Lichen planus 0.5–2 mg per kg daily PO as a single AM dose








Lupus erythematosus, subacute






Morphea 0.5–2 mg per kg daily PO as a single AM dose


Necrobiotic xanthogranuloma



Nummular eczema 0.5–2 mg per kg daily PO as a single AM dose for 7–21 days


Otitis externa 0.5–2 mg per kg daily PO as a single AM dose for 7–21 days


Prednisone. Dermatologic indications and dosage (Continued)



Prednisone 477

P

Papular mucinosis 0.5–2 mg per kg daily PO as a single AM dose





Pemphigus vulgaris 1–2 mg per kg daily PO as a single AM dose

1–2 mg per kg daily PO as a single AM dose

Pityriasis lichenoides 0.5–2 mg per kg daily PO as a single AM dose



1 mg per kg PO as a single AM dose for 7–14 days


Post-herpetic neuralgia prophylaxis

1 mg per kg PO daily for 14–21 days Not indicated

Pruritic papules and plaques of pregnancy


Not applicable

Psoriasis 1 mg per kg PO as a single AM dose for 7–14 days



1 mg per kg PO as a single AM dose 1 mg per kg PO as a single AM dose

Sarcoidosis 20–40 mg daily PO for 2–3 months, followed by slow taper to 10 mg every other day for up to 1 year

10–30 mg daily PO for 2–3 months, followed by slow taper to 5 mg every other day for up to 1 year

Scabies 1 mg per kg PO as a single AM dose for 7–14 days


Schnitzler syndrome 1 mg per kg PO daily for 14–21 days 1 mg per kg PO daily for 14–21 days

Seborrheic dermatitis 0.5–2 mg per kg daily PO as a single AM dose for 7–21 days


Serum sickness 0.5–1 mg per kg PO daily for 7–21 days

0.5–1 mg per kg PO daily for 7–21 days

Stasis dermatitis 1 mg per kg PO as a single AM dose for 7–14 days


Stevens-Johnson syndrome

1 mg per kg PO daily for 14–21 days 1 mg per kg PO daily for 14–21 days


1 mg per kg PO daily as a single AM dose

1 mg per kg PO daily as a single AM dose

Temporal arteritis 1–2 mg per kg PO daily as a single AM dose

1–2 mg per kg PO daily as a single AM dose

Urticaria 1 mg per kg PO daily for no longer than 21 days; to be used only in severe, recalcitrant disease

1 mg per kg PO daily for no longer than 21 days; to be used only in severe, recalcitrant disease

Vogt-Koyanagi-Harada syndrome - iritis






478 Pregnancy mask

Contraindications/precautionsHypersensitivity to drug class or compo-nent; systemic fungal infection; caution inpatients with congestive heart failure, sei-zure disorder, hypertension, diabetes melli-tus, tuberculosis; osteoporosis; impairedliver function

ReferencesWilliams LC, Nesbitt LT (2001) Update on system-

ic glucocorticosteroids in dermatology. Der-matologic Clinics 19(1):63–77

Pregnancy mask

� Melasma

Pregnancy-associated autoimmune disease


Premalignant fibroepithelial tumor

� Fibroepithelioma of Pinkus

Pressure alopecia


Pressure sore

� Decubitus ulcer

Pressure ulcer

� Decubitus ulcer

Prickle cell carcinoma

� Squamous cell carcinoma

Prickly heat

� Miliaria







Xerotic eczema 0.5–1 mg per kg daily PO as a single AM dose for 7–10 days





Progressive lipodystrophy 479

P

Primary adrenal insufficiency


Primary cutaneous neuroendocrine carcinoma

� Merkel cell carcinoma

Primary hemochromatosis

� Hemochromatosis

Primary hypertrophic osteoarthropathy

� Pachydermoperiostosis

Primary localized cutaneous amyloidosis

� Lichen amyloidosis

Primary Raynaud’s

� Raynaud’s disease

Primary varicella

� Varicella

Principen

� Ampicillin

Progressive and recurring dermatofibroma


Progressive capillary hemangioma

� Tufted angioma

Progressive cardiomyopathic lentiginosis



Synonym(s)Progressive partial lipodystrophy; Barra-quer-Simons syndrome; acquired partiallipodystrophy; cephalothoracic dystrophy;acquired progressive lipodystrophy

DefinitionDisorder characterized by progressive andsymmetric loss of subcutaneous fat

PathogenesisMay be associated with glomerulonephri-tis, low C3 levels, and the presence of a C3nephritic factor


480 Progressive partial lipodystrophy

Clinical manifestationOnset between 5 and 15 years of age; slow,insidious loss of subcutaneous fat, initiallylimited to the face, sometimes extending tothe upper portion of the body, giving thepatient a cachetic appearance

Differential diagnosisHIV-associated lipodystrophy; Cockaynesyndrome; generalized lipodystrophiessuch as Berardinelli-Seip syndrome; Wernersyndrome; hypothalamus tumor

TherapySubcutaneous fat injections from unaf-fected areas; temporal muscle flaps; sili-cone filling material

ReferencesKetterings C (1988) Lipodystrophy and its treat-

ment. Annals of Plastic Surgery 21(6):536–543

Progressive partial lipodystrophy


Progressive septic granulomatosis


Progressive symmetric keratoderma

Synonym(s)Erythrokeratodermia progressiva symmet-rica

DefinitionHereditary keratoderma with slowly pro-gressive, symmetric, and well-definedhyperkeratotic plaques

PathogenesisAutosomal dominant trait; defect in loric-rin gene or in an unknown locus onchromosome 1

Clinical manifestationWell demarcated, red, scaly plaques, dis-tributed with almost perfect symmetry onthe head, extremities, palms, soles, and but-tocks; chest and abdomen usually spared;onset during the first year of life or shortlythereafter, progressing for a few years, andthen stablilizing; some cases remit sponta-neously

Differential diagnosisErythrokeratodermia variabilis; Giroux-Barbeau erythrokeratodermia with ataxia;Greither disease; erythrokeratolysis hiema-lis; ichthyosis linearis circumflexa; psoria-sis; lupus erythematosus; lamellar ichthyo-sis; gyrate erythema; atopic dermatitis

TherapyKeratolytics such as alpha hydroxy acids;acitretin

ReferencesGray LC, Davis LS, Guill MA (1996) Progressive

symmetric erythrokeratodermia. Journal of the American Academy of Dermatology 34(5 Pt 1):858–859

Progressive systemic sclerosis

Synonym(s)Systemic sclerosis; scleroderma; systemicconnective tissue disease; diffuse systemicsclerosis


Proliferating systematized endotheliosis 481

P

DefinitionMultisystem connective tissue disorder,characterized by vasomotor disturbances,fibrosis of the skin, subcutaneous tissue,muscles, and internal organs

PathogenesisImmunologic system abnormality and vas-cular changes; increased collagen produc-tion or disturbances in its degradation,causing excessive collagen deposition in tis-sues

Clinical manifestationSkin: areas of hyperpigmentation alternat-ing with hypopigmentation; overall appear-ance of tanned skin persists long after sunexposure; telangiectasias on face, neck, andperiungual areas; skin of the hands some-times edematous or indurated early, latersclerotic stage where skin is tight and shiny,with a loss of hair, decreased sweating, andloss of ability to make a skin fold; starts dis-tally on the fingers; any area of the bodyultimately may be involved; calcinosis onthe fingers and extremities; reduced oralaperture (microstomia) from perioralinvolvementEars, nose and throat: xerostomia andxerophthalmia; vascular changes – Ray-naud phenomenon triggered by cold, smok-ing, or emotional stress; infarction and drygangrene sometimes results from severevasospasmMusculoskeletal system: arthralgias andmorning stiffness sometimes mimickingother systemic autoimmune diseases; handand joint function may decline from skintightening; acroosteolysis (i.e., resorptionor dissolution of the distal end of the pha-lanx) sometimes occurs; flexion contrac-turesNeurologic system: trigeminal neuralgia;carpal tunnel syndromeRespiratory system: dry rales, indicatingfibrosisEsophageal sphincter incompetenceGastrointestinal system: reflux; esophagitisBarrett metaplasia; candidiasis; water-melon stomach or gastric vascular antralectasia; primary biliary cirrhosis; malab-

sorption; atrophy of smooth muscle andfibrotic changes leading to decreased peri-stalsis throughout the gastrointestinal tractRenal system: renal failure; cardiac involve-ment: indicates poor prognosis; pericardialeffusions with cor pulmonale; conductionabnormalities; infiltrative cardiomyopathy

Differential diagnosisMorphea; linear scleroderma; bleomycin-induced scleroderma; toxic oil syndrome;porphyria cutanea tarda; digital sclerosis ofdiabetes mellitus; radiation exposure;intestinal obstruction from other causes;infiltrative cardiomyopathy from othercauses; eosinophilia-myalgia syndrome;chronic graft versus host disease

TherapyD-penicillamine: 250–1500 mg per day POdivided into 2 or 3 doses; methotrexate

ReferencesSapadin AN, Fleischmajer R (2002) Treatment of

scleroderma. Archives of Dermatology 138(1): 99–105

Proliferating endotheliosis


Proliferating pilar tumor

� Pilar tumor

Proliferating systematized endotheliosis



482 Proliferating trichilemmal cyst

Proliferating trichilemmal cyst

� Pilar tumor

Protein energy malnutrition

� Marasmus

Protocoproporphyria


Protoporphyria


Protothecosis, cutaneous

Synonym(s)Infection by achlorophillic algae

DefinitionInfection caused by algae of the genus Pro-totheca

PathogenesisUsually caused by Prototheca wickerhamii;wide variety of aqueous sources, includinglakes, streams, ponds; host immunsuppres-sion is a risk factor

Clinical manifestationHistory of trauma (e.g., abrasion, cut) toskin and subsequent exposure to contami-nated water; extremities most commonsites of involvement; ill-defined plaque ornodule, often with verrucous surface; bul-lae with rupture, drainage, and crusting

Differential diagnosisBacterial pyoderma; orf; milker’s nodule;anthrax; atypical mycobacterial infection;nocardiosis; deep fungal infection, such ascryptococcosis, chromomycosis, coccidio-idomycosis, or North American blastomy-cosis

TherapyCombination of tetracycline and amphoter-icin B: 0.5 mg per kg IV daily for 1–6 weeks�; ketoconazole; itraconazole

ReferencesThiele D, Bergmann A (2002) Protothecosis in hu-

man medicine. International Journal of Hy-giene & Environmental Health 204(5-6):297–302

Prurigo gestationis

� Prurigo of pregnancy


Synonym(s)Pruritus of pregnancy; prurigo gestationis;early-onset prurigo of pregnancy; papulardermatitis of pregnancy; pruritic folliculitisof pregnancy

DefinitionDisorder occurring in the second half ofpregnancy, characterized by discrete,crusted papules located predominantly overthe extensor aspects of the limbs, shoul-ders, and abdomen

PathogenesisPruritus gravidarum variant may be associ-ated with intrahepatic cholestasis, perhapsresulting from elevated estrogen and pro-gesterone levels thought to interfere withthe liver's ecretion of bile salts


Pruritus ani 483

P

Clinical manifestationPruritus with papules produced by scratch-ing, usually occurring in last trimester ofpregnancy; may have jaundice withcholestasis

Differential diagnosisScabies; insect bite reaction; impetigo her-petiformis; pemphigoid gestationis (herpesgestationis); pruritic urticarial papules andplaques of pregnancy

TherapyCorticosteroids, topical, medium potency

ReferencesVaughan Jones SA, Hern S, Nelson-Piercy C, Seed

PT, Black MM (1999) A prospective study of 200 women with dermatoses of pregnancy cor-relating clinical findings with hormonal and immunopathological profiles. British Journal of Dermatology 141(1):71–81

Pruritic folliculitis of pregnancy



Synonym(s)Polymorphic eruption of pregnancy;toxemic erythema of pregnancy; toxemicrash of pregnancy; late-onset prurigo ofpregnancy; PUPPP

DefinitionDermatosis of pregnancy characterized byintensely pruritic red papules and plaquesarising late in the third trimester

PathogenesisMay be related to increased skin distension

Clinical manifestationErythematous urticarial papules andplaques of the trunk and extremities, mostnotably in striae; periumbilical area spared;usually arises in third trimester, particu-larly in first pregnancy; no fetal effects;resolves within weeks of partuition

Differential diagnosisUrticaria; erythema multiforme; cholesta-sis of pregnancy; impetigo herpetiformis;herpes gestationis; papular dermatitis ofpregnancy; prurigo gestationis; viral exan-them; drug eruption

TherapyCorticosteroids, topical, high potency;prednisone for severe flares

ReferencesAronson IK, Bond S, Fiedler VC, Vomvouras S,

Gruber D, Ruiz C (1998) Pruritic urticarial pa-pules and plaques of pregnancy: clinical and immunopathologic observations in 57 patients. Journal of the American Academy of Dermatol-ogy 39(6):933–939

Pruritus ani

Synonym(s)Anal itching

Pruritic urticarial papules and plaques of pregnancy. Erythematous papules and plaques on the abdomen of a pregnant woman, with accentuation in striae


484 Pruritus gravidarum

DefinitionPruritus involving the area around the anus

PathogenesisFinal common pathway for multiple incit-ing factors, including: perfumes, chemicals,or dye on toilet paper; moisture from sweator diarrhea; certain foods, including caf-feine, chocolate, beer, nuts, dairy products,and spicy foods; infections or infestationssuch as pinworm infestation, candidiasis, orgenital warts; hemorrhoids; recent antibi-otic use

Clinical manifestationPruritus, often without obvious dermato-sis; may have erythema with or withoutexudate, depending upon inciting factors

Differential diagnosisNeurodermatitis; drug hypersensitivityreaction; anal carcinoma; contact dermati-tis

TherapyCareful attention to hygiene: gentle butthorough rectal cleansing after bowel move-ments; drying powders; sitz baths; corticos-teroids, topical, low potency; cotton placedover anal orifice to minimize fecal leakage;lubricating cream or lotion to perianal areatwice daily

ReferencesNagle D, Rolandelli RH (1996) Primary care office

management of perianal and anal disease. Pri-mary Care Clinics in Office Practice 23(3):609–620

Pruritus gravidarum


Pruritus of pregnancy


Pseudo Hodgkin’s disease


Pseudo Kaposi’s sarcoma

� Acroangiodermatitis� Granuloma gluteale infantum

Pseudo Turner syndrome

� Noonan’s syndrome

Pseudo Ullrich-Turner syndrome


Pseudochromhidrosis plantaris

� Black heel

Pseudocolloid lip mucous membrane sebacous milia


Pseudocolloid of the buccal mucosa



Pseudohypoparathyroidism 485

P

Pseudocolloid of the lips



Synonym(s)Pseudofolliculitis of the beard; pili incar-nati; folliculitis barbae traumatica; shavingbumps; razor bumps

DefinitionForeign body reaction from ingrown curlyhair characterized by papules and pustulesin the beard area

PathogenesisTightly curved hair from a recent shavebriefly surfaces from the skin and reenters ashort distance away, producing foreignbody reaction; transfollicular penetrationoccurs when the sharp tip of hair piercesthe follicle wall without emerging from skin

Clinical manifestationFlesh-colored or erythematous papule withcentral hair shaft, seen in shaved areas adja-cent to the follicular ostia; pustules andabscess formation from secondary infec-tion; postinflammatory hyperpigmenta-tion, scarring, and keloid formation afterchronic involvement

Differential diagnosisFolliculitis; acne vulgaris; tinea barbae;acne keloidalis; sarcoidosis; granulomaannulare; rosacea

TherapyShaving techniques: stop shaving for at least3–4 weeks; clean beard with face cloth, wetsponge, or soft-bristled toothbrush with amild soap for several minutes before shav-ing; shave with 3-headed, rotary electricrazor with heads slightly off skin surface;shave in a slow circular motion; use chemi-

cal depilatories; laser destruction of hairfollicles in affected areas

ReferencesPerry PK, Cook-Bolden FE, Rahman Z, Jones E,

Taylor SC (2002) Defining pseudofolliculitis barbae in 2001: a review of the literature and current trends. Journal of the American Acade-my of Dermatology 46(2 Suppl Understand-ing):S 113–119

Pseudofolliculitis of the beard


Pseudohomozygous familial hypercholesterolemia

� Phytosterolemia

Pseudohypoparathyroidism

Synonym(s)Albright hereditary osteodystrophy

DefinitionHereditary condition which resembleshypoparathyroidism, but caused by a lackof response to parathyroid hormone ratherthan a deficiency of the hormone

PathogenesisMolecular defects in the gene (GNAS1)encoding the alpha subunit of the stimula-tory G protein; unresponsivieness of appro-priate organs to the actions of parathyroidhormone

Clinical manifestationSoft tissue calcifications; hypocalcemia;brachydactyly; dimples may replace knuck-


486 Pseudolymphoma

les on affected digits; short stature; mentalretardation; basal ganglia calcifications; cat-aracts; tetany; hyperphosphatemia andhypocalcemia; normal parathyroid hor-mone levels

Differential diagnosisDystrophic calcification; hypoparathy-roidism; secondary hyperparathyroidism;autoimmune polyglandular syndromes

TherapyCalcium 1200 mg PO per day; 1-alpha-hydroxylated vitamin D metabolites 250 mgPO per day; surgical excision of sympto-matic soft tissue calcifications

ReferencesBastepe M, Juppner H (2000) Pseudohypoparath-

yroidism. New insights into an old disease. En-docrinology and Metabolism Clinics of North America 29(3):569–589

Pseudolymphoma

Synonym(s)Lymphocytoma cutis; cutaneous lym-phomatous hyperplasia; lymphadenosisbenigna cutis; cutaneous lymphoplasia;pseudolymphoma of Spiegler-Fendt; Spieg-ler-Fendt sarcoid

DefinitionGroup of disorders with a common trait ofresponse to stimuli resulting in a lymphom-atous-appearing but benign accumulationof inflammatory cells

PathogenesisMost cases with unknown inciting agent;some known agents: tattoo dye, jewelry,insect bite reaction, medications, folliculi-tis, trauma, vaccinations, irritants, cutane-ous infection

Clinical manifestationB cell variant: one or several firm, red-to-violaceous nodules, from one to severalcentimeters in diameterT-cell variant: broad, erythematous patchesand/or plaques

Differential diagnosisInsect bite; inflamed epidermoid cyst; gran-uloma faciale; foreign body granuloma;granuloma annulare; granulomatous rosa-cea; Jessner’s lymphocytic infiltration; lym-phoma; metastasis; basal cell carcinoma;squamous cell carcinoma; Merkel cell carci-noma; actinic reticuloid; lymphomatoidpapulosis

TherapyTriamcinolone 3–4 mg per ml intralesional;superficial radiation; surgical excision; liq-uid nitrogen cryotherapy

ReferencesGilliam AC, Wood GS (2000) Cutaneous lym-

phoid hyperplasias. Seminars in Cutaneous Medicine & Surgery 19(2):133–141

Pseudolymphoma of Spiegler-Fendt

� Pseudolymphoma

Pseudomonas folliculitis


Pseudopapilledema



Pseudopyogenic granuloma 487

P

Pseudopelade

Synonym(s)Pseudopelade of Brocq; Brocq pseudopel-ade

DefinitionEnd stage or clinical variant of variousforms of scarring alopecia

PathogenesisLinked to underlying disease, such as lupuserythematosus or lichen planus

Clinical manifestationRandomly distributed, irregularly shapedareas of scarring alopecia of scalp (“foot-prints in the snow”), often with hypopig-mentation and slight atrophy; few hairssometimes remain in otherwise completelybald and scarred plaque; no clinical evi-dence of inflammation

Differential diagnosisLupus erythematosus; lichen planus; follic-ular degeneration syndrome; alopeciaareata; post-traumatic alopecia; folliculitisdecalvans; lichen sclerosus; androgeneticalopecia


ReferencesHeadington JT (1996) Cicatricial alopecia. Der-


Pseudopelade of Brocq

� Pseudopelade

Pseudoporphyria

Synonym(s)Drug-induced bullous photosensitivity;therapy-induced bullous photosensitivity

DefinitionBullous photosensitivity disorder mimick-ing porphyria cutanea tarda, withoutdemonstrable porphyrin abnormalities

PathogenesisAssociated with ingestion of certain medi-cations and with hemodialysis

Clinical manifestationIncreased skin fragility; erythema; tensebullae and erosions on sun-exposed skin,without hypertrichosis or sclerodermoidskin changes; variant mimicking erythro-poietic protoporpria in children onnaproxen for juvenile rheumatoid arthritis

Differential diagnosisPorphyria cutanea tarda; erythropoieticprotoporphyria; epidermolysis bullosaacquisita; bullous pemphigoid; bullouslupus erythematosus

TherapyDiscontinued use of offending agent;reduced sun exposure until blistering erup-tion has cleared

ReferencesGreen JJ, Manders SM (2001) Pseudoporphyria.

Journal of the American Academy of Dermatol-ogy 44(1):100–108

Pseudopyogenic granuloma



488 Pseudosarcoma

Pseudosarcoma


Pseudosarcomatous dermatofibroma


Pseudosarcomatous reticulohistiocytoma


Pseudoxanthoma elasticum

Synonym(s)Systematized elastorrhexis; Grönblad-Strandberg syndrome

DefinitionHereditary connective tissue disease char-acterized by symptoms and signs second-ary to progressive calcification and frag-mentation of elastic fibers in the skin, ret-ina, and cardiovascular system

PathogenesisDominant and recessive types, withunknown gene defects; may be related toabnormal glycosaminoglycan secretion,causing calcification and fragmentation ofelastic fibers

Clinical manifestationSkin findings: symmetrical, small, yellowpapules, coalescing into plaques in a linearpattern, giving affected skin “pluckedchicken” appearance; first noted on the lat-eral neck and later involving antecubital

fossae, axillae, popliteal fossae, inguinaland periumbilical areas, oral, vaginal andrectal mucosa; with disease progression,skin sometimes becomes soft, lax, wrin-kled, and hangs in folds; elastosis per-forans serpiginosa may coexistOcular findings: bilaterally symmetricalangioid streaks of retina, noted severalyears after onset of cutaneous lesions; mayhave to retinal hemorrhages; progressiveloss of central visionCardiovascular findings: usually the lastlesions to be recognized; peripheral pulsesoften severely diminished; hypertension;coronary artery disease causes angina pec-toris and subsequent myocardial infarc-tion; mitral valve prolapse; gastrointestinalhemorrhage, usually gastric in origin; lesscommonly, hemorrhaging occurs in uri-nary tract or cerebrovascular system

Differential diagnosisMarfan syndrome; Ehlers-Danlos syn-drome; Buschke-Ollendorff syndrome;localized acquired cutaneous pseudoxan-thoma elasticum; penicillamine therapy;actinic damage to the lateral neck

TherapySurgical correction of lax skin; diet andexercise to minimize risks associated withcardiovascular disease

ReferencesSherer DW, Sapadin AN, Lebwohl MG (1999)

Pseudoxanthoma elasticum: an update. Der-matology 199(1):3–7

Psoriasis

Synonym(s)None

DefinitionChronic inflammatory skin disorder char-acterized by scaly, red papules and plaquesdistributed over extensor body surfaces andthe scalp


Psoriasis 489

P

PathogenesisMultifactorial, with genetic factors andenvironmental triggers, including infec-tions (e.g., HIV and streptococcal), smok-ing, UV light, medications such as lithium,and emotional factors; T-cell immunologicreaction causes epidermal hyperprolifera-tion

Clinical manifestationPlaque variant: sharply demarcated, redpapules and plaques, with silvery-whitescale, most often located on scalp, trunk,and limbs, with predilection for extensorsurfaces, such as the elbows and knees; ten-dency toward bilateral symmetry; develop-ment of lesions in traumatized skin (Koeb-ner phenomenon); lesions encircled by apaler peripheral zone (Woronoff ring); nailswith pitting, onycholysis, subungual hyper-keratosis, irregular and brown nail bed dis-coloration (oil-drop sign)Pustular variant: may occur after with-drawal of systemic corticosteroids; patientsometimes systemically ill with fever, leuco-cytosis; generalized or patchy erythemastudded with pustules in annular or non-specific configuration; flexural and ano-genital accentuation; may also appear ontrunk or extremities and rarely on face; may

involve one or few digits only (acrodermati-tis continua of Hallopeau)Guttate variant: may follow infection, mostcommonly streptococcal; multiple, dis-crete, salmon pink, scaly, droplike papules,beginning on trunk and proximal extremi-ties and spreading to face, ears, and scalp;palms and soles rarely affected; all variantsmay include psoriatic arthritis

Differential diagnosisPlaque and guttate variants: pityriasis rubrapilaris; seborrheic dermatitis; tinea cor-poris; lupus erythematosus; pityriasisrosea; syphilis; lichen planus; parapsoria-sis; pityriasis lichenoides; cutaneous T-celllymphoma; nummular eczemaPustular variant: subcorneal pustular der-matosis; acute generalized exanthematouspustulosis; septicemia; generalized atopicand/or seborrheic dermatitis; dyshidroticeczema; contact dermatitis; autosensitiza-tion reaction; vesicular dermatophyte infec-tion

TherapyTopical therapy: corticosteroids, topical,super potent; coal tar 1–5 % gel appliednightly; anthralin; calcipotriene; tazaroteneScalp therapy: corticosteroids, topical, highpotency in foam or lotion; anti-seborrheicshampoo used daily; phenol/saline lotionapplied to wet scalp nightly under showercap; UVB phototherapy; photochemother-apy; excimer laser therapySystemic therapy: methotrexate; acitretin;cyclosporine; thioguanine; mycophenolatemofetil; hydroxyurea; alefacept – 7.5 mg IMweekly for 12 weeks

References Lui H (2002) Phototherapy of psoriasis: update

with practical pearls. Journal of Cutaneous Medicine & Surgery 6(3 Suppl):17–21

Silvis N (2001) Antimetabolites and cytotoxic drugs. Dermatologic Clinics 19(1):105–118

Tremblay JF, Bissonnette R (2002) Topical agents for the treatment of psoriasis, past, present and future. Journal of Cutaneous Medicine & Sur-gery 6(3 Suppl):8–11

Psoriasis. Scaly, red plaques of the feet


490 Psychogenic purpura

Psychogenic purpura


� Gardner-Diamond syndrome

PTEN hamartoma tumor syndrome


Pulmonic stenosis

� Watson syndrome

Punctate keratoderma

Synonym(s)None

DefinitionCondition characterized by histologicpunctate thickening of the stratum cor-neum, possibly as part of a generalized con-dition or a disorder primarily involving thehands and feet

ReferencesRatnavel RC, Griffiths WA (1997) The inherited

palmoplantar keratodermas. British Journal of Dermatology 137(4):485–490

Punctate porokeratosis

� Porokeratosis

PUPPP


Purpura

DefinitionSuperficial hemorrhage into the skin, up to1 cm in diameter



Purpura annularis telangiectodes


Purpura autoerythrocytica


Purpura en cocarde avec oedema



Pyoderma gangrenosum 491

P

Purpura fulminans

DefinitionRapidly developing, generalized purpura,associated with severe disturbance of thecoagulation system, usually with dissemi-nated intravascular coagulation

ReferencesDarmstadt GL (1998) Acute infectious purpura

fulminans: pathogenesis and medical manage-ment. Pediatric Dermatology 15(3):169–183

Pustular perifolliculitis

� Acne necrotica

Pyoderma

� Ecthyma

Pyoderma faciale

� Rosacea


Synonym(s)None

DefinitionDisorder characterized by sudden onset ofrapidly expanding cutaneous ulceration,often in patients with preexisting systemicdisease such as rheumatoid arthritis,inflammatory bowel disease, or myeloge-nous leukemia

PathogenesisMay be a hypersensitivity reaction to anti-genic stimuli

Clinical manifestationClassic subtype: small, red papule or pus-tule evolving into deep ulceration; oftenarising at site of minor trauma, with viola-ceous undermined border; occurs mostcommonly on legs, but may be seen on anyskin surface, including around stoma sites(peristomal pyoderma gangrenosum);intraoral ulcerated plaques (pyostomatitisvegetans), primarily in patients withinflammatory bowel diseaseAytical subtype: vesiculopustular compo-nent only at the border, with erosion orsuperficial ulceration; most often occurs ondorsal aspect of hands, extensor surface offorearms or facePyoderma vegetans subtype: crusted,hyperplastic plaques without deep ulcera-tion, similar to that seen in pyostomatitisvegetans; all subtypes may be associatedwith underlying polyarthritis, inflamma-tory bowel disease, myelogenous leukemia,or monoclonal gammopathy

Differential diagnosisVasculitis; Wegener’s granulomatosis; spi-der bite reaction; squamous cell carcinoma;sporotrichosis; orf; milker’s nodule; herpessimplex virus infection (particularly inimmunosuppressed patient); antiphosphol-ipid antibody syndrome; anthrax; vascularinsufficiency; acute febrile neutrophilic der-matosis; North American blastomycosis;traumatic ulceration, including factitial dis-ease; tuberculosis; syphilis

TherapyPrednisone; steroid-sparing agents: azathi-oprine; dapsone; cyclophosphamide; myco-phenolate mofetil; cyclosporine; hydrocol-loid dressings

ReferencesPowell FC, O'Kane M (2002) Management of pyo-

derma gangrenosum. Dermatologic Clinics 20(2):347–355


492 Pyoderma vegetans

Pyoderma vegetans

� Pyoderma gangrenosum

Pyogenic granuloma

Synonym(s)Lobular capillary hemangioma; granulomapyogenicum; granuloma telangiectaticum

DefinitionVascular skin tumor characterized by soli-tary, glistening, red papule or nodule thatbleeds easily and may ulcerate

PathogenesisUnknown

Clinical manifestationRapidly enlarging, bright red, friable, poly-poid papule or nodule, sometimes sponta-neously bleeding, eroding, or ulcerating;occurs most commonly on gingiva, lips,nasal mucosa, face, and distal extremities;may develop multiple recurrent lesionsafter prior attempts at removal; when

occurring in pregnancy, found along themaxillary intraoral mucosal surface, butany intraoral, perioral, and nonoral tissuemay be involved; associated with indinaviruse

Differential diagnosisMelanoma; squamous cell carcinoma;Kaposi’s sarcoma; atypical fibroxanthoma;excess granulation tissue; glomus tumor;capillary hemangioma; angioendotheli-oma; angiolymphoid hyperplasia; angiosar-coma; hemangioendothelioma; intravascu-lar angiomatosis; tufted hemangioma

TherapySurgical excision; destruction by electro-desiccation and curettage

ReferencesPark YH, Houh D, Houh W (1996) Subcutaneous

and superficial granuloma pyogenicum. Inter-national Journal of Dermatology 35(3):205–206

Pyostomatitis vegetans

� Pyoderma gangrenosum


Q

Quintan fever

�

Trench fever


R

Rabbit fever

�

Tularemia

Radiation dermatitis

Synonym(s)

Radiodermatitis

Definition

Skin disorder at the site of exposure to X-irradiation

Pathogenesis

Radiation effects on stem cells, preventingrenewal of aging or injured cells


Acute variant: occurs after single or fewlarge doses of radiation; erythema andedema within 24 hours of dosing; second-ary, progressive erythema 3–6 days afterirradiation, with vesicles and bullae if doseis sufficiently high; desquamation followedby postinflammatory hyperpigmentation,often with atrophyChronic variant: atrophy, telangiectasia,and dryness, often with skin tethering tounderlying tissue; ulceration in center ofradiation scar, often 1–2 years after com-plete healing of skin following radiationtherapy


Contact dermatitis; basal cell carcinoma;squamous cell carcinoma; traumatic ulcera-tion; decubitus ulceration; erythema abigne; retiform purpura (discontinuouslivedo reticularis)

Therapy

Biopsy of suspicious ulcerations to rule outskin cancer; protective padding to mini-mize trauma

References

Porock D, Nikoletti S, Kristjanson L (1999) Man-agement of radiation skin reactions: literature review and clinical application. Plastic Surgical Nursing 19(4):185–192

Radiodermatitis

�

Radiation dermatitis

Ramsay Hunt syndrome

�

Herpes zoster

Raspberry lesion

�



496 Rat-bite fever

Rat-bite fever

Synonym(s)

Streptobacillary fever

;

Haverhill fever

;

epidemic arthritic erythema

;

spirillaryfever

;

Sodoku

Definition

Systemic febrile illness transmitted in thesecretions of the mouth, nose, or urine ofan infected rodent, often by rat bite

Pathogenesis

Caused by two different organisms, Strepto-bacillus moniliformis and Spirillum minus;acquired through contact with urine or oralor conjunctival secretions from an infectedanimal, usually after bite


Variant caused by Streptobacillus monili-formis: fever, chills, headache, and musclepain, usually occurring within 10 days ofexposure, followed within 3 days by diffuseerythematous eruption, primarily in thedistal extremities; ulceration at site of bite;one or several large joints sometimesbecome swollen, red, and painful; occa-sional splenomegalyVariant caused by Spirillum minus (Sod-oku): red or purple plaques; previouslyhealed wound at site of bite sometimesreactivate and ulcerate; rare joint involve-ment


Viral exanthem; drug eruption; rickettsio-sis; legionellosis; leptospirosis; Lyme dis-ease

Therapy

Aqueous penicillin G: 1.2–2.4 million unitsper day IV for 7 days, followed by penicillinV 500 mg PO for 7 days

�

; tetracycline forpenicillin-allergic patients

References

Cunningham BB, Paller AS, Katz BZ (1998) Rat bite fever in a pet lover. Journal of the Ameri-

can Academy of Dermatology 38(2 Pt 2):330–332

Raynaud disease

�

Raynaud’s disease

Raynaud syndrome

�

Raynaud’s disease

Raynaud’s disease

Synonym(s)

Raynaud’s syndrome

;

Raynaud disease

;

Raynaud syndrome

;

primary Raynaud’s

Definition

Disorder characterized by paroxysmalvasospasm (Raynaud’s phenomenon), with-out association with another illness

Pathogenesis

Abnormal blood flow to affected areas;abnormal recovery from cold stimuli;decreased blood flow may occur fromincreased blood viscosity or pathologic ves-sel constriction


Paroxysmal color changes: white, blue, andthen red; affected body part usuallychanges colors at least twice during an epi-sode; completely reversible; rare extremeischemia of the affected body part mayresult in necrosis and digital ulceration


Raynaud’s phenomenon associated withunderlying disease, such as scleroderma,lupus erythematosus, dermatomyositis,rheumatoid arthritis, viral hepatitis or neo-plastic disease; chilblains; frostbite; Buerger


Reactive perforating collagenosis 497

R

disease; paroxysmal nocturnal hemoglob-inuria; peripheral arterial occlusive dis-ease; acrocyanosis; carpal tunnel syn-drome; thoracic outlet syndrome

Therapy

Nifedipine: 30–90 mg PO daily; losartan –50 mg PO daily

References

Wigley FM (2002) Clinical practice. Raynaud’s phenomenon. New England Journal of Medi-cine 347(13):1001–1008

Raynaud’s phenomenon

Definition

Reversible constriction of peripheral arteri-oles in response to a variety of stimuli, mostcommonly caused by exposure to cold orstressful circumstances

References

Wigley FM (2002) Clinical practice. Raynaud’s phenomenon. New England Journal of Medi-cine 347(13):1001–1008

Raynaud’s syndrome

�

Raynaud’s disease

Razor bumps

�


Reactive angioendotheliomatosis

�


Reactive inflammatory systematized angioendotheliomatosis

�



Synonym(s)

Acquired perforating disease

;

collagenomaperforant verruciforme

;

acquired reactiveperforating dermatosis

Definition

Inherited and acquired dermatosis in whichthe skin eliminates keratotic debris andaltered collagen fibers by the transepider-mal route

Pathogenesis

Minor skin trauma causes focal damage tocollagen, followed by elimination of the dis-rupted collagen through the epidermis


Flesh-colored, dome-shaped papules with acentral keratotic plug occurring at sites ofminor trauma; most commonly found onthe extensor surfaces of the limbs and dorsaof the hands; linear distribution (Koebnerphenomenon); scarring occurs with healing


Kyrle’s disease; perforating folliculitis; elas-tosis perforans serpiginosa; prurigo nodu-laris; Ferguson-Smith type of keratoacan-thoma

Therapy

Tretinoin 0.025% cream; adapalene 0.1%gel; photochemotherapy; isotretinoin;emollients to control pruritus


498 Reactive perforating elastosis

References

Faver IR, Daoud MS, Su WP (1994) Acquired reac-tive perforating collagenosis. Report of six cas-es and review of the literature. Journal of the American Academy of Dermatology 30:575–580

Reactive perforating elastosis

�


Recessive dystrophic epidermolysis bullosa

�


Recurrent aphthous stomatitis

�

Aphthous stomatitis

Recurrent aphthous ulcers

�

Aphthous stomatitis

Recurrent granulomatous dermatitis with eosinophilia

�


Recurrent painful bruising

�

Gardner-Diamond syndrome

Recurring digital fibroma of childhood

�


Refsum disease

Synonym(s)

Heredopathia atactica polyneuritiformis

Definition

Neurocutaneous syndrome characterizedbiochemically by phytanic acid accumula-tion in plasma and tissues, resulting inperipheral polyneuropathy, cerebellarataxia, retinitis pigmentosa, and ichthyosis

Pathogenesis

Autosomal recessive trait; mutation in thephytanoyl-CoA hydroxylase gene causesdefective peroxisomal alpha-oxidation ofphytanic acid; tissue accumulation of thisfatty acid, which derives from exogenoussources (mainly from dietary plant chloro-phyll and from animal tissues)


Skin findings: variable ichthyosiformplaques over lower trunk and extremitiesNeurologic/ocular findings: partial, inter-mittent, sensorimotor polyneuropathy; cat-aracts; nystagmus; concentric visual fieldconstriction; sensorineural deafness; cere-bellar ataxia; skeletal defects; cardiomyopa-thy


Ichthyosis vulgaris; lamellar ichthyosis; X-linked ichthyosis; Sjögren-Larsson syn-drome; chronic and intermittent polyneuri-tis; relapsing infectious polyneuritis; mito-chondrial myopathies; acute intermittentporphyria; toxin exposure; hereditarymotor neuropathies


Reiter syndrome 499

R

Therapy

Phytanic acid-free diet

�

; plasmapheresis;alpha hydroxy acids

References

Wills AJ, Manning NJ, Reilly MM (2001) Refsum's disease. QJM 94(8):403–406

Regressing atypical histiocytosis

�

Cutaneous CD30+ (Ki-1) anaplastic large-cell lymphoma

Reiter disease

�

Reiter syndrome

Reiter syndrome

Synonym(s)

Reiter disease

;

Fiessinger-Leroy-Reiter syn-drome

;

Fiessinger-Leroy syndrome

;

arthritis urethritica

;

blennorrheal idio-pathic arthritis

Definition

Multisystem disorder characterized by pso-riasis-like plaques, balanitis, keratoderma,conjunctivitis, urethritis, arthritis, andspondylitis, often after episode of urethritisor dysentery

Pathogenesis

Probable immunologic hypersensitivityreaction to microorganism; genetic factorsfar more common in men; HLA B-27 com-mon haplotype in affected individuals


Diarrhea and dysenteric syndrome orsymptoms of urethritis prior to other find-

ings; circinate balanitis with circular orgyrate white plaques growing centrifugallyover glans penis; conjunctivitis with intensered, conjunctival injection; joint symptomsresembling rhematoid arthritis, but asym-metrical and often involving single joint;knee and tarsal joints and sacroiliac regionmost commonly involved; psoriasiformcutaneous lesions; palms and soles mostcommonly involved with keratotic papules,plaques, and pustules; keratoderma blenor-rhagica, with painful, keratotic papules andplaques; distal involvement with painfuland erosive lesions in the tips of the fingersand toes, with pustules; nail dystrophy; redmacules and plaques, diffuse erythema,erosions, and bleeding on oral and pharyn-geal mucosae; circinate lesions on tongueresembling geographic tongue; commonsyndrome in patients with HIV disease


Psoriasis; pityriasis rubra pilaris; lichenplanus; lupus erythematosus; dermatomy-ositis; Behçet’s disease; arthritis associatedwith gonococcal disease, rheumatoidarthritis; septic arthritis; scabies; mycosisfungoides; subcorneal pustulosis of Sned-don-Wilkinson; atopic dermatitis; acuteexanthematic pustulosis; other causes oferythroderma

Therapy

Topical therapy: corticosteroids, topical,super potent; coal tar 1–5 % gel appliednightly, anthralin; calcipotriene; tazaroteneScalp therapy: corticosteroids, topical, highpotency foam or lotion; anti-seborrheicshampoo used daily; phenol/saline lotionapplied to wet scalp nightly under showercap; UVB phototherapy; photochemother-apy; excimer laser therapySystemic therapy: methotrexate; acitretin;cyclosporine; thioguanine; mycophenolatemofetil; hydroxyurea

References

Hughes RA, Keat AC (1994) Reiter's syndrome and reactive arthritis: a current view. Seminars in Arthritis & Rheumatism 24(3):190–210


500 Relapsing febrile nodular nonsuppurative panniculitis

Relapsing febrile nodular nonsuppurative panniculitis

�


Relapsing febrile nonsuppurative nodular panniculitis

�


Relapsing fever

Synonym(s)

Tick-borne relapsing fever

;

louse-bornerelapsing fever

Definition

Acute infectious disease transmitted byticks or lice, caused by several species of thegenus Borrelia

Pathogenesis

Louse-borne spirochetes transmitted eitherby bite of louse or by inoculation of lousefeces; tick-borne spirochetes enter hostblood stream after bite


Acute onset of illness with fever, headache,chills, sweats, myalgias, arthralgia; dizzi-ness, nausea, and vomiting; dry mucousmembranes; petechiae on the trunk andextremities; photophobia and conjunctivalinjection; scleral icterus; nonproductivecough; pleuritic pain; epistaxis; blood-tinged sputum


Lyme disease; Rocky Mountain spottedfever; leptospirosis; Colorado tick fever;trench fever; rat bite fever; dengue fever

Therapy

Tetracycline

�

; doxycycline; erythromycin

References

Rahlenbeck SI, Gebre-Yohannes A (1995) Louse-borne relapsing fever and its treatment. Tropi-cal & Geographical Medicine 47(2):49–52

Shapiro ED (1997) Tick-borne diseases. Advances in Pediatric Infectious Diseases 13:187–218


Synonym(s)

Polychondropathy

;

systemic chondromala-cia

;

chronic atrophic polychondritis

Definition

Episodic inflammatory disease of cartilagi-nous structures, predominantly those of theear, nose, and laryngotracheobronchial tree

Pathogenesis

Probably immune-mediated


Erythema and edema overlying inflamedcartilaginous structures; vasculitis of skinand other organs; sudden onset of unilat-eral or bilateral auricle pain, swelling, andredness, sparing the lobules; nonerosive,seronegative inflammatory polyarthritis;acute nasal chondritis with pain and feel-ing of fullness over nasal bridge; episodicinflammation of the uveal tract, conjuncti-vae, sclerae, and cornea; respiratory tractchondritis; auricular chondritis, with sud-den hearing loss, tinnitus, nausea, vomit-ing, nystagmus, and vertigo; cardiovascularstructural changes


Cellulitis; polyarteritis nodosa; chondro-dermatitis nodularis helicis; rheumatoidarthritis; Cogan syndrome; infectious peri-chondritis; MAGIC syndrome; trauma;syphilis; chronic external otitis; auricularcalcification from trauma; Addison disease;diabetes or hyperthyroidism


Reticulate acropigmentation of Kitamura 501

R

TherapyPrednisone�; steroid-sparing agents: dap-sone, azathioprine, methotrexate, cyclo-phosphamide, cyclosporine, methotrexate

ReferencesTrentham DE, Le CH (1998) Relapsing polychon-

dritis. Annals of Internal Medicine 129(2):114–122

REM syndrome

� Reticular erythematous mucinosis

Rendu-Osler syndrome


Respiratory scleroma

� Rhinoscleroma

Reticular erythematous mucinosis

Synonym(s)REM syndrome; round cell erythematosus

DefinitionDermal mucinosis presenting as erythema-tous, infiltrated reticulated plaques

PathogenesisMay be related to abnormal mucopolysac-charide production from populations ofFXIIIa+/HAS2+ dermal dendrocytes

Clinical manifestationAsymptomatic or slightly pruritic, ery-thematous, infiltrated papules, either iso-

lated or coalescing into plaques, in the mid-line of the back or chest; exascerbation withsun exposure

Differential diagnosisGeneralized myxedema; pretibial myxe-dema; scleredema; scleromyxedema; papu-lar mucnosis; focal mucinosis; cutaneousmucinosis of infancy; nevus mucinosis; alo-pecia mucinosa; lupus erythematosus

TherapyHydroxychloroquine�; pulse dye laser

ReferencesCohen PR, Rabinowitz AD, Ruszkowski AM,

DeLeo VA (1990) Reticular erythematous mu-cinosis syndrome: review of the world litera-ture and report of the syndrome in a prepubertal child. Pediatric Dermatology 7(1):1–10

Reticulate acropigmentation of Kitamura

Synonym(s)Kitamura’s reticulate acropigmentation;Kitamura’s acropigmentatio reticularis

DefinitionReticulate, lentigo-like pigmenation of thedorsal aspects of the hands

PathogenesisAutosomal dominant inheritance; exactdefect unknown

Clinical manifestationNet-like hyperpigmentation, with atrophy,of the dorsal aspects of the hands; pigmen-tation at other sites as the patient ages; pal-mar pits may be associated

Differential diagnosisAcromelanosis progressiva; acropigmenta-tion of Dohi; universal acquired melanosis


502 Reticulate pigmented anomaly

TherapyNone

ReferencesSchnur RE, Heymann WR (1997) Reticulate hy-

perpigmentation. Seminars in Cutaneous Med-icine & Surgery 16(1):72–80

Reticulate pigmented anomaly

Synonym(s)Dowling-Degos disease; dark dot disease;Dowling Degos Ossipowski disease

DefinitionProgressive. acquired pigment disorder,characterized by flexural, pigmented reticu-late macules, and comedone-like papuleson the back and neck

PathogenesisAutosomal dominant trait; unknown genedefect

Clinical manifestationFlexural pigmentation with onset fromchildhood to adult life; brownish-blackcolor with steely-gray or navy hues; some-times stippled in shades of brown; palpableplaques from secondary lichenification;margins may have punctate pigmentedcomedones; occasional speckled maculesinvolving the dorsum of the hands, proxi-mal nail folds, or scrotum

Differential diagnosisCarney’s syndrome; acanthosis nigricans;confluent and reticulate papillmatosis ofGougerot-Carteaud; Kitamura reticulateacropigmentation; Haber syndrome; Galli-Galli disease


ReferencesAmichai B, Grunwald AM, Bergman R (1997)

Guess what? European Journal of Dermatology 7(6): 465–466

Reye tumor

� Infantile digital fibromatosis

Rhabdomyoblastoma

� Rhabdomyosarcoma

Rhabdomyosarcoma

Synonym(s)Malignant rhabdomyoma; myosarcoma;sarcoma botryoides; rhabdomyoblastoma

DefinitionMalignant mesenchymal tumor with stri-ated muscle differentiation

PathogenesisUnknown

Clinical manifestationMass lesion, often in infancy or early child-hood, usually involving head and neckregion, genitourinary tract, or deep soft tis-sues of the extremities

Differential diagnosisRhabdomyoma; lymphoma; liposarcoma;malignant fibrous histiocytoma

TherapySurgical excision, followed by radiationand/or chemotherapy�


Rhinoscleroma 503

R

ReferencesWomer RB, Pressey JG (2000) Rhabdomyosarco-

ma and soft tissue sarcoma in childhood. Cur-rent Opinion in Oncology 12(4):337–344

Rhagades

DefinitionLinear fissures of the skin, especially on theanus or at the corner of the mouth, some-times due to syphilis

ReferencesParish JL (2000) Treponemal infections in the

pediatric population. Clinics in Dermatology 18(6):687–700

Rheumatoid nodule

DefinitionFirm, non-tender, freely-movable, subcuta-neous nodule, usually in periarticular loca-tion, seen with rheumatoid arthritis

ReferencesSwezey RL (1997) The management of rheuma-

toid nodules. American Journal of Orthopedics 26(2):73

Rheumatoid vasculitis


Rhinoscleroma

Synonym(s)Mikulicz disease; respiratory scleroma;scleroma

DefinitionChronic granulomatous disease of the noseand other structures of the upper respira-tory tract, resulting from infection by thebacterium Klebsiella rhinoscleromatis

PathogenesisCaused by infection from bacterium Kleb-siella rhinoscleromatis, contracted by directinhalation of droplets or contaminatedmaterial

Clinical manifestationAffects nasal cavity, nasopharynx, larynx,trachea, and bronchiGranulomatous (hypertrophic) stage: nasalmucosa is bluish red and granular, with for-mation of rubbery nodules or polyps in thenose; epistaxis; deformity and destructionof the nasal cartilage (Hebra nose); thick-ened soft palate, with erythematous,crusted papules or nodulesSclerotic stage: nodules replaced by fibroustissue, leading to scarring and stenosis

Differential diagnosisVerrucous carcinoma; leprosy; Wegener’sgranulomatosis; leishmaniasis; lymphoma(lethal midline granuloma); actinomycosis;syphilis; yaws; sarcoidosis; Langerhans cellhistiocytosis; tuberculosis; actinomycosis;syphilis; leprosy; histoplasmosis; blastomy-cosis; paracoccidioidomycosis; sporotricho-sis; rhinospiridiosis

Rheumatoid nodule. Subcutaneous nodule on the elbow


504 Rhinosporidiosis

TherapyTetracycline�; ciprofloxacin

ReferencesLenis A, Ruff T, Diaz JA, Ghandour EG (1988) Rhi-

noscleroma. Southern Medical Journal 81(12):1580–1582

Rhinosporidiosis

Synonym(s)None

DefinitionInfectious disease characterized by slow-growing, tumorlike mass, arising in thenasal mucosa or ocular conjunctivae,caused by aquatic protozoan parasite, Rhi-nosporidium seeberi

PathogenesisCaused by aquatic protozoan parasite, Rhi-nosporidium seeberi, after local traumaticinoculation with the organism, in peoplebathing or working in stagnant water

Clinical manifestationUnilateral nasal obstruction or epistaxis;other symptoms: local pruritus, coryza withsneezing, rhinorrhea, and postnasal dis-charge with cough; soft, pink-to-deep-red,sessile or pedunculated polyps on the noseor eye; skin lesions begin as papillomas andgradually become verrucous

Differential diagnosisRhinoscleroma; condyloma acuminatum;nasal polyp; mucocele; squamous cell carci-noma


ReferencesElgart ML (1996) Unusual subcutaneous infec-

tions. Dermatologic Clinics 14(1):105–111

Rhus dermatitis


Rice-field fever

� Leptospirosis

Richner-Hanhart syndrome

� Tyrosinemia II

Rickettsemia

� Typhus

Rickettsialpox

Synonym(s)Gamasid rickettsiosis; vesicular rickettsio-sis

DefinitionSelf-limited, zoonotic, febrile illness, causedby rickettsial organism, characterized bypapulovesicular skin rash at the site of themite bite

PathogenesisCausative agent: Rickettsia akari; vector:colorless mite, Allodermanyssus san-guineus, found on mice and other rodents

Clinical manifestationBite, preceding febrile illness and produc-ing red papule with central vesicle sur-rmounting it; lesion dries with black


Rifampin 505

R

eschar; prodrome of high fever, lasting for aweek, with occasional remissions in themorning; generalized exanthem; lesions ontongue, buccal mucosa, and pharynx; mildconstitutional symptoms

Differential diagnosisVaricella; scrub typhus; hand-foot-mouthdisease; Boutonneuse fever; viral exanthem

TherapyTetracycline; doxycycline; ciprofloxacin

ReferencesBoyd AS (1997) Rickettsialpox. Dermatologic

Clinics 15(2):313–318

Riehl melanosis


Riehl’s melanosis

Synonym(s)Riehl melanosis; pigmented cosmetic der-matitis; pigmented contact dermatitis;melanosis faciei feminae; erythrodermaexfoliativa recidivans faciei; lichen ruberplanus cum pigmentatione

DefinitionPigmented contact dermatitis of the face,most commonly caused by sensitizingchemicals in cosmetics

PathogenesisType IV allergic reaction; basement mem-brane damaged by allergic reaction, caus-ing melanin from damaged cells to fall intoupper dermis to be ingested by macro-phages; ultraviolet light may be a factor;inciting agents: formaldehyde, brilliant lakered R, musk ambrette, optical brightenersand analine dyes

Clinical manifestationSudden onset of diffuse or patchy brownpigmentation of cheeks and forehead;severe cases may be black, purple, or blue-black; reticular pigment patterning; ery-thematous macules or papules

Differential diagnosisBerloque dermatitis; phytophotodermati-tis; melasma; polymorphous light erup-tion; lupus erythematosus; lichen planus;postinflammatory hyperpigmentation


ReferencesHori Y, Takayama O (1988) Circumscribed dermal

melanoses. Classification and histologic fea-tures. Dermatologic Clinics 6(2):315–326

Rifampin

Trade name(s)Rifadin; Rimactane


Drug classRifamycin

Mechanism of actionInhibits bacterial RNA synthesis by inhibit-ing DNA-dependent RNA polymerase

Dosage form150 mg, 300 mg capsule


Common side effectsCutaneous: pruritus, urticaria or othereruptionsGastrointestinal: abdominal pain, nausea,vomiting, diarrheaLaboratory: elevated liver enzymes


506 Right Guard Sport

Miscellaneous: reddish-orange body fluids,stained contact lensesNeurologic: dizziness, ataxia, headache

Serious side effectsBone marrow: thrombocytopenia, leukope-niaGastrointestinal: hepatotoxicityRenal: renal failure, interstitial nephritis

Drug interactionsDigoxin; chloramphenicol; warfarin; phe-nobarbital; phenytoin; ketoconazole; theo-phylline; verapamil; cyclosporine; corticos-teroids; oral contraceptives; dapsone; sulfo-nylureas

Contraindications/precautionsHypersensitivity to drug class or compo-nent; caution in patients with hepatic insuf-ficiency

ReferencesTsankov NK, Kamarashev JA (1993) Rifampin in

dermatology. International Journal of Derma-tology 32(6):401–406

Right Guard Sport


Riley-Smith syndrome


Ringed keratolysis

� Pitted keratolysis

Ringworm

� Tinea corporis

Ringworm of the beard

� Sycosis barbae

Ringworm of the face

� Tinea faciei

Rifampin. Dermatologic indications and dosage


Cutaneous tuberculosis

10 mg per kg PO daily, divided into 2 doses


Leprosy, multibacillary disease

600 mg daily PO for 3 years 10–20 mg per kg daily PO for 3 years

Leprosy, paucibacillary disease

300 mg PO twice daily for 3 months 10–20 mg per kg PO daily for 3 months

Staphylococcal pyoderma, to eliminate carrier state

600 mg PO daily for 7 days 10–20 mg per kg daily PO for 7 days


Rocky Mountain spotted fever 507

R

Ringworm of the feet

� Tinea pedis

Ringworm of the groin

� Tinea cruris

Ringworm of the scalp

� Tinea capitis

Robert-Unna syndrome


Robles’ disease

� Filariasis


Synonym(s)Tick fever; spotted fever; tick typhus; NewWorld spotted fever; Sao Paulo fever

DefinitionTick-borne rickettsial disease, character-ized by fever, rash, and constitutional signsand symptoms

PathogenesisCaused by R rickettsii, rickettsial organismtransmitted from tick to human during

feeding; proliferates in the endothelial lin-ing, causing intravascular thrombi; vasculi-tis leads to small vessel occlusion and tis-sue necrosis

Clinical manifestationPresents within 1 week of tick bite; pro-drome of fever, headache, myalgias; skinand mucous membrane changes: confluentmacular and papular eruption on wristsand ankles; spreads centripetally to trunkand proximal extremities and palms andsoles; eruption becomes petechial after afew days; conjunctival suffusion; perior-bital edema, especially in children; photo-phobiaCardiovascular system: myocarditis; brady-cardia; arrhythmias; occasional hypoten-sion; congestive heart failure secondary tomyocarditisPulmonary system: pulmonary edema insevere cases; pneumonitisGastrointestinal system: anorexia; abdomi-nal pain and tenderness; jaundice in severecases; hepatomegaly and splenomegaly;diarrheaMusculoskeletal system: myalgia, especiallyin the legs, abdomen, and back; diffusearthralgias; edema of the dorsum of handsand feetCentral nervous system: restlessness andirritability; altered mental status; menin-goencephalitis; cranial neuropathies; paral-ysis; ataxia; meningismus

Differential diagnosisDengue fever; babesiosis; ehrlichiosis;mononucleosis; leptospirosis; Lyme dis-ease; malaria; meningococcemia; bacterialsepsis; toxic shock syndrome; tularemia;other rickettsial infections; allergic vasculi-tis; Brill-Zinsser disease; drug hypersensi-tivity; atypical measles; rubeola; drug erup-tion

TherapyDoxycycline�; chloramphenicol:adult dose: 500 mg IV divided into 4 dosesper day for 7 days;


508 Rodent ulcer

pediatric dose: 50 mg per kg PO dividedinto 4 doses for 7 days and for at least 48hours after defervescence

ReferencesSexton DJ, Kaye KS (2002) Rocky Mountain spot-

ted fever. Medical Clinics of North America 86(2):351–360

Rodent ulcer


Romberg-Perry syndrome

� Morphea

Romberg’s facial hemiatrophy

� Morphea

Rosacea

Synonym(s)Acne rosacea

DefinitionDisorder characterized by facial flushingand a spectrum of clinical signs includingerythema, telangiectasia, and inflamma-tory papules and pustules

PathogenesisGenetic component; preferentially occurs inthose with constitutive facial flushing; prob-ably related to the local release of vasoac-

tive substances; exascerbated by local heatfrom hot drinks, alcohol, spicy foods andtemperature changes

Clinical manifestationBackground of facial flushing; erythemaand telangiectasia over the cheeks and fore-head; inflammatory papules and pustules,predominantly over the nose, forehead, andcheeks; extra-facial involvement over theneck and upper chest; prominent seba-ceous glands with development of thick-ened and disfigured nose (rhinophyma)Ocular variant: conjunctival injection,chalazion, and episcleritisGranulomatous variant (lupus miliaris dis-seminata faciei): inflammatory, erythema-tous or flesh-colored papules distributedsymmetrically across the upper face, partic-ularly around the eyes and nose

Differential diagnosisSeborrheic dermatitis; lupus erythemato-sus; polymorphous light eruption; tineafaciei; acne vulgaris; perioral dermatitis;folliculitis; lupus vulgaris; carcinoid syn-drome

TherapyTetracycline; minocycline; doxycycline;metronidazole; azelaic acid; tretinoin;isotretinoin; surgical therapy: permanenttelangiectasia: 585-nm pulsed dye laser; rhi-nophyma: mechanical dermabrasion; CO2laser peel

Rosacea. Red papules on the cheek


Rothman-Makai syndrome 509

R

ReferencesRebora A (2002) The management of rosacea.

American Journal of Clinical Dermatology 3(7):489–496

Rosacea-like dermatitis


Rose gardener's disease

� Sporotrichosis

Roseola

Synonym(s)Roseola infantum; exanthem subitum; sixthdisease

DefinitionChildhood exanthematous disease causedby Human Herpesvirus-6 (HHV-6)

PathogenesisMain cause HHV-6B; in primary infection,replication of the virus in leukocytes andsalivary glands; early invasion of the cen-tral nervous system, causes seizures andother CNS complications

Clinical manifestationMost primary infections asymptomatic;typical presentation: 9–12-month-old childwith abrupt onset of high fever (40ºC), last-ing for 3 days with nonspecific complaints;febrile seizures may occur; rapid deferves-cence of fever occurring with onset of pinkmorbilliform exanthem composed of eitherdiscrete, small, pale pink papules or ablanchable exanthem, lasting 2 days; enan-them (Nagayama's spots) with erythema-tous papules on the mucosa of the soft pal-ate and base of the uvula

Differential diagnosisOther viral exanthems, including mononu-cleosis; rubeola and rubella; scarlet fever;meningococcemia; dengue fever; medica-tion reaction

TherapyAntipyretic therapy such as acetaminophen

ReferencesBlauvelt A (2001) Skin diseases associated with

human herpesvirus 6, 7, and 8 infection. Jour-nal of Investigative Dermatology. Symposium Proceedings 6(3):197–202

Roseola infantum

� Roseola

Rothman-Makai syndrome

Synonym(s)Lipogranulomatosis subcutanea; adipone-crosis subcutanea; lipophagic panniculitisof childhood

DefinitionPanniculitis of children, characterized bysubcutaneous nodules without systemicsigns or symptoms

PathogenesisMay be a variant of Weber-Christian dis-ease; mechanism of disease unknown

Clinical manifestationWell-demarcated, somewhat painful, sym-metrical subcutaneous nodules, most oftenon lower extremities and trunk; atrophy inlesions of lipophagic panniculitis variant

Differential diagnosisThrombophlebitis; vasculitis; sarcoidosis;alpha-1 antitrypsin deficiency panniculitis;polyarteritis nodosa; eosinophilic fasciitis;eosinophilic myalgia syndrome; erythema


510 Rothmund-Thomson syndrome

induratum; erythema nodosum; leukemia;lipodermatosclerosis; lymphoma; pancre-atic panniculitis; poststeroid panniculitis;scleroderma panniculitis; cytophagic histi-ocytic panniculitis; Sweet’s syndrome


ReferencesRequena L, Sanchez Yus E (2001) Panniculitis.

Part II. Mostly lobular panniculitis. Journal of the American Academy of Dermatology 45(3):325–361

Rothmund-Thomson syndrome

Synonym(s)Poikiloderma congenitale

DefinitionHereditary disorder characterized by multi-system abnormalities and early photosensi-tivity, resulting in poikiloderma

PathogenesisAutosomal recessive trait; genetic defect onchromosome 8

Clinical manifestationIrregular erythema of the skin progressingto poikiloderma with atrophy, telangiecta-sia, hyperpigmentation, and hypopigmen-tation; sparse hair; premature canities; dys-trophic or atrophic nails; acral hyperkera-totic lesions on elbows, knees, hands, andfeet; distinctive facies with frontal bossing,saddle nose, and micrognathia; short stat-ure; sexual abnormalities; cataracts; dentalabnormalities

Differential diagnosisBloom syndrome; lupus erythematosus;erythropoietic protoporphyria; Werner’ssyndrome progeria; Fanconi’s anemia; acro-geria; Cockayne syndrome; xeroderma pig-mentosus; Mendes da Costa syndrome

TherapySun protection�; pulse dye laser therapy fortelangiectases

ReferencesVennos EM, Collins M, James WD (1992) Roth-

mund-Thomson syndrome: review of the world literature. Journal of the American Acad-emy of Dermatology 27(5 Pt 1):750–762

Round cell erythematosus

� Reticular erythematous mucinosis

Rubella

Synonym(s)German measles; three day measles

DefinitionContagious viral infection with mild symp-toms associated with eruption and lym-phadenopathy

PathogenesisRNA virus classified as a Rubivirus in theTogaviridae family

Clinical manifestationSpread by nasal droplet infection; incuba-tion period of 14–19 days, with onset of rashusually on the 15th day; disease contagiousfrom a few days before to 5–7 days after theappearance of the exanthem; most conta-gious when rash is erupting; may have noprodrome in children, with rash being firstmanifestation; in adults, fever, sore throat,and rhinitis may occur; discrete macules onthe face that spread to the neck, trunk, andextremities, with coalescence into plaques;exanthem lasts 1–3 days, first leaving theface; nonspecific enanthem (Forscheimer'sspots) of pinpoint red macules andpetechiae visible over the soft palate anduvula just before or with the exanthem;


Rubeola morbilli 511

R

generalized tender lymphadenopathyinvolving all nodes, but most striking in thesuboccipital, postauricular, and anteriorand posterior cervical nodes; joint symp-toms may occur in adults; congenitalrubella syndrome in infants whose motherscontract the disease during the first trimes-ter: purpura at birth, low birth weight,small head size, lethargy, irritabilitiy, deaf-ness, seizures, developmental delay, mentalretardation

Differential diagnosisJuvenile rheumatoid arthritis; rubeola;other viral exanthems; scarlet fever; Kawa-saki disease; drug eruption

TherapyNone; isolation for 7 days after onset of theeruption

ReferencesBullens D, Smets K, Vanhaesebrouck P (2000)

Congenital rubella syndrome after maternal reinfection. Clinical Pediatrics 39(2):113–116

Rubeola

Synonym(s)Measles; rubeola morbilli; rubeola measles

DefinitionAcute, contagious, viral disease character-ized by distinct red lesions in the mouthfollowed by a generalized eruption

PathogenesisMeasles virus infects respiratory epithe-lium; transmitted via respiratory droplets;replication in lymph nodes leads toviremia; infection of endothelial cellsensues, causing enanthem (Koplik spots);infection of epithelial cells leads to skineruption

Clinical manifestationIncubation period from 7–14 days (average10–11 days); communicable just before thebeginning of prodromal symptoms, untilapproximately 4 days following the onset ofthe exanthem; prodrome of cough, coryza,conjunctivitis, fever, photophobiaEnanthem (Koplik spots): blue-white spotssurrounded by red halo; appear on buccalmucosa opposite the premolar teeth; pre-date exanthem by 24–48 hours and lastapproximately 2–4 days.Exanthem: begins on the fourth or fifth dayafter onset of symptoms; appears as slightlyelevated papules beginning on face andbehind the ears and spreading to trunk andextremities within 24–36 hours; initial colordark red, slowly fading to purplish hue, andthen to yellow/brown lesions with finescale, over the following 5–10 days

Differential diagnosisOther viral exanthems, such as rubella,enterovirus, echovirus, cytomegalovirusinfection, primary HIV disease; brucello-sis; drug eruption; Kawasaki disease


� Rubella

ReferencesOmer MI (1999) Measles: a disease that has to be

eradicated. Annals of Tropical Paediatrics 19(2):125–134

Rubeola measles

� Rubeola

Rubeola morbilli

� Rubeola


512 Rubinstein syndrome

Rubinstein syndrome

� Rubinstein-Taybi syndrome

Rubinstein Taybi broad thumb-hallux syndrome

� Rubinstein-Taybi syndrome

Rubinstein-Taybi syndrome

Synonym(s)Rubinstein syndrome; broad thumb-halluxsyndrome

DefinitionGenetic multisystem disorder character-ized by broad thumbs and great toes, char-acteristic facies, and mental retardation

PathogenesisPossible autosomal dominant inheritance insome families; gene on the short arm (p) ofchromosome 16 (16p13.3); may be caused bypoint mutation or deletion in gene involvedin regulation of CREB binding protein

Clinical manifestationSkin changes: one or capillary hemangi-omas or nevus flammeus lesions on fore-head, neck nape, and/or back; cafe au laitspots; keloid formation; hypertrichosis;ingrown finger- or toenails.Systemic changes: growth retardation;delayed bone age; mental retardation;craniofacial dysmorphism (includinghypertelorism, broad nasal bridge, and“beak-shaped” nose); abnormally broadthumbs and great toes; breathing and swal-lowing difficulties; malformations of theheart, kidneys, urogenital system, and/orskeletal system

Differential diagnosisSaethre-Chotzen syndrome; Trisomy 13 syn-drome; Cornelia de Lange syndrome


ReferencesDe Silva B (2002) What syndrome is this? Ruben-

stein-Taybi syndrome. Pediatric Dermatology 19(2):177–179

Rudimentary polydactyly

� Supernumerary digit

Runaround abscess

� Paronychia

Runaround infection

� Paronychia

Ruvalcaba-Myhre-Smith syndrome


Ruvalcaba-Myhre syndrome



S

Saethre-Chotzen syndrome

�

Acrocephalosyndactyly

Sailor’s neck

�

Actinic elastosis

Salivosudoriparous syndrome

�


Salmon patch

Synonym(s)

Stork bite

;

angel’s kiss

;

nevus simplex

;

erythema nuchae

Definition

Vascular malformation, present in infancy,consisting of ectatic capillaries

Pathogenesis

Possible persistent fetal circulatory pattern


Pink-to-red macule or patch on the nape ofthe neck, glabella, forehead, upper eyelid,or nasolabial region; present at birth inabout 40% of cases


Hemangioma; Sturge-Weber syndrome;child abuse; insect bite reaction

Therapy

No therapy indicated

References

Mallory SB (1991) Neonatal skin disorders. Pedi-atric Clinics of North America 38(4):745–761

Salmonellosis

Synonym(s)

Typhoid fever

;

paratyphoid fever

Definition

Infection caused by bacteria in the genusSalmonella, usually contracted by eatingcontaminated food

PART19.MIF Friday, October 31, 2003 12:22 PM

514 San Joaquin Valley fever

Pathogenesis

Infection caused by salmonellae, gram-neg-ative, rod-shaped bacteria of the familyEnterobacteriaceae; most common sourcesof bacteria: beef, poultry, eggs


Skin signs: light red papules (rose spots)occurring in crops on trunk during secondto fourth week of illness; erythema nodo-sum, Sweet’s syndrome, pustular dermati-tis and generalized erythroderma (ery-thema typhosum)Gastrointestinal signs: loose stool or waterydiarrhea; abdominal pain; mild hepat-osplenomegaly


Viral gastroenteritis; shigellosis; ingestionof preformed toxins (“food poisoning”);campylobacter infection; cryptosporidio-sis; cyclospora infection; escherichia coliinfection; listeriosis; vibrio infection

Therapy

Antibiotics only for patients with severedisease or those at high risk of invasive dis-ease: ciprofloxacin; amoxicillin

�

Typhoid fever

References

Stutman HR (1994) Salmonella, shigella, and campylobacter: common bacterial causes of in-fectious diarrhea. Pediatric Annals 23(10):538–543

San Joaquin Valley fever

�

Coccidioidomycosis

Sandworm disease

�


Sanfilippo syndrome

Synonym(s)

Mucopolysaccharidosis type III-A

;

muco-polysaccharidosis type III-B

;

mucopoly-saccharidosis type III-C

Definition

Inherited metabolic storage disease from adeficiency of either heparan sulfate sulfami-dase,

N-

acetyl-alpha-D-glucosaminidase,acetyl-CoA alpha-glucosamide

N-

acetyl-transferase, or

N-

acetyl-alpha-D-glu-cosamine-6-sulfatase

Pathogenesis

Autosomal recessive trait; deficiency ofeither heparan sulfate sulfamidase, or

N-

acetyl-alpha-D-glucosaminidase, or acetyl-CoA alpha-glucosamide

N-

acetyltrans-ferase, or

N-

acetyl-alpha-D-glucosamine-6-sulfatase, resulting in accumulation ofmucopolysaccharides in the lysosomes ofthe cells in the connective tissue


Onset of symptoms from age 2–6 years;organs most involved: bone, viscera, con-nective tissue, and brain; regression of psy-chomotor development and neurologicsigns, including severe mental retardation,hyperactivity, autistic features, and behav-ioral disorders; thickened facial features;coarse hair; hirsutism; genu valgum; shortneck; progressive deterioration and death,usually before age 20 years


Hunter syndrome; Hurler syndrome; Scheiesyndrome; Gaucher’s disease; Niemann-Pick disease

Therapy

None

References

Yogalingam G, Hopwood JJ (2001) Molecular ge-netics of mucopolysaccharidosis type IIIA and


Say syndrome 515

S

IIIB: Diagnostic, clinical, and biological impli-cations. Human Mutation 18(4):264–281

Sao Paulo fever

�


Sarcoidosis

Synonym(s)

Angiolupoid sarcoid

;

Besnier-Boeck-Schau-mann disease

;

Boeck's sarcoid

Definition

Chronic multisystem disease, characterizedby noncaseating epithelioid granulomas

Pathogenesis

May result from exposure of a geneticallysusceptible host to specific environmentalagents, such as infectious organisms, alu-minium, zirconium, talc, pine tree pollen,and clay, that the immune system is unableto effectively clear


Skin: asymptomatic, red-brown maculesand papules commonly involving the face,periorbital, nasolabial folds, extensor sur-faces of extremities; round-to-oval, red-brown-to-purple, infiltrated plaques, thecenter of which may be atrophic; non-tender, firm, oval, flesh-colored or viola-ceous nodules on extremities or trunk(Darier-Roussy sarcoidosis); inflitration ofscarsPulmonary system: involvement in mostpatients; dyspnea; dry cough; chest tight-ness or painLymphatic system: palpable lymph nodesOcular involvement: anterior uveitis, asso-ciated with fever and parotid swelling (uve-oparotid fever)

Neurologic system: central nervous systeminvolvement sometimes fatal; seventh cra-nial nerve palsy most frequent finding; mis-cellaneous findings: myocardial involve-ment, arthritis, proximal muscle weakness,renal failure


Tuberculosis; lymphoma; pseudolym-phoma; foreign body granuloma; drug reac-tion; granuloma annulare; granulomafaciale; lichen planus; lupus erythematosus;leprosy; syphilis; psoriasis; tinea corporis;necrobiosis lipoidica

Therapy

Cutaneous involvement: triamcinolone3 mg per ml intralesionalSevere, recalcitrant disease: methotrexate;azathioprine; hydroxychloroquineSymptomatic systemic disease: pred-nisone

�

References

Vourlekis JS, Sawyer RT, Newman LS (2000) Sar-coidosis: developments in etiology, immunolo-gy, and therapeutics. Advances in Internal Medicine 45:209–257

Sarcoma botryoides

�

Rhabdomyosarcoma

Savill’s syndrome

�


Say syndrome

�

Barber-Say syndrome


516 Scabies

Scabies

Synonym(s)

Seven-year itch

Definition

Contagious infestation of the skin by arach-nid mite Sarcoptes scabiei, var hominis

Pathogenesis

Causative organism is mite, Sarcoptes sca-biei; disease spreads through direct andprolonged contact between hosts; possibletransmission through fomites, such asinfected bedding or clothing, but less likely;delayed type IV hypersensitivity reaction tomites, eggs, or scybala (packets of feces)which causes intense pruritus


Intense pruritus, particularly at night;slightly elevated, pink-white, linear, curved,or s-shaped line (burrow), located inwebbed spaces of fingers, flexor surfaces ofwrists, elbows, axillae, belt line, feet, andscrotum in men and areolae in women; bur-rows on the palms and soles in infants; vesi-cles; red papules on penile shaftNodular variant: pink, tan, brown, or rednodules lasting for weeksCrusted (Norwegian) variant: occurs inimmunocompromised and institutional-ized patients; minimally pruritic, hyperk-

eratotic, crusted plaques over large areas;nail dystrophy; scalp lesions


Atopic dermatitis; dermatitis herpeti-formis; pityriasis lichenoides; lichen pla-nus; insect bite reaction; contact dermati-tis; psoriasis; ecthyma; impetigo; xeroticeczema; transient acantholytic dermatosis;linear IgA bullous dermatosis; seborrheicdermatitis; erythroderma from othercauses such as Sézary syndrome and pem-phigus foliaceus; Langerhans cell histiocy-tosis; fiberglass dermatitis; dyshidroticeczema; pityriasis rosea; animal scabies;pediculosis; delusions of parasitosis; meta-bolic pruritus

Therapy

Permethrin; ivermectin; prednisone forsevere symptoms

References

Wendel K, Rompalo A (2002) Scabies and pedicu-losis pubis: an update of treatment regimens and general review. Clinical Infectious Diseases 35(Suppl 2):S146–151

Scalded skin syndrome

�

Staphylococcal scalded skin syn-drome

Scalp and head syndrome

�

Adams-Oliver Syndrome

Scalp cyst

�

Pilar cyst

Scabies.

Scaly and fissured papules and plaques in the finger web spaces


Schamberg’s progressive pigmented purpura 517

S

Scarlatina

�

Scarlet fever

Scarlet fever

Synonym(s)

Scarlatina

Definition

Bacterial infection caused by toxin-produc-ing group-A beta hemolytic streptococci

Pathogenesis

Eruption caused by erythemogenic toxin asconsequence of local production of inflam-matory mediators and alteration of thecutaneous cytokines


Abrupt onset of fever, headache, vomiting,malaise, chills, and sore throat, with rashappearing after 1–4 days; exudative tonsilli-tis a common site of infection; mucousmembranes usually bright red; scatteredpetechiae and small, red papules on softpalate; during first days of infection, whitemembrane coating on tongue throughwhich edematous, red papillae protrude(white strawberry tongue); after whitemembrane sloughs, tongue red with promi-nent papillae (red strawberry tongue);

exanthem consisting of fine red, punctatepapules, appearing within 1–4 days follow-ing the onset of illness; first appear onupper trunk and axillae and then general-ize, with accentuation in flexural areas; mayappear more intense at dependent sites andsites of pressure, such as the buttocks; sand-paper feel to affected skin; transverse areasof hyperpigmentation with petechiae in theaxillary, antecubital, and inguinal areas(Pastia lines); flushed face with circumoralpallor; rash fades with fine desquamationafter 4–5 days


Viral exanthem, including rubella, rubeola,fifth disease; toxic shock syndrome; Kawa-saki syndrome; lupus erythematosus; drugreaction

Therapy

Penicillin VK

�

; benzathine penicillin G;penicillin allergy – cephalexin, erythromy-cin

References

Chiesa C, Pacifico L, Nanni F, Orefici G (1994) Re-current attacks of scarlet fever. Archives of Pediatrics & Adolescent Medicine 148(6):656–660

Scarring pemphigoid

�


Schamberg disease

�


Schamberg’s progressive pigmented purpura

�


Scarlet fever.

Bright red tongue with prominent papillae


518 Scheie syndrome

Scheie syndrome

Synonym(s)

Mucopolysaccharidosis type I-H/S

;

muco-polysaccharidosis type I-S

;

Hurler-Scheiesyndrome

Definition

Inherited metabolic storage disease arisingfrom a deficiency of alpha-L-iduronidase

Pathogenesis

Autosomal recessive trait; deficiency ofalpha-L-iduronidase, which results in accu-mulation of mucopolysaccharides in thelysosomes of the cells in the connective tis-sue


Onset of symptoms from age 2–4 years;signs and symptoms similar to those ofHurler syndrome, but milder, with slowerprogression; lichenified, dry, thick skinwith diminished elasticity; increased pig-mentation on the dorsum of the hands;sclerodermalike changes; hypertrichosis ofthe extremities; pale colored hair; mildskeletal deformation and deformity of thehands; growth sometimes normal; aorticstenosis or regurgitation sometimespresent; hepatosplenomegaly; intelligenceusually normal


Hurler syndrome; Hunter syndrome; Gau-cher’s disease; Niemann-Pick diseae; osteo-genesis imperfecta

Therapy

None

References

Schiro JA, Mallory SB, Demmer L, Dowton SB, Luke MC (1996) Grouped papules in Hurler-Scheie syndrome. Journal of the American Academy of Dermatology 35(5 Pt 2):868–870

Schenck's disease

�

Sporotrichosis

Schilder-Addison syndrome

�


Schnitzler syndrome

Synonym(s)

Schnitzler’s syndrome

Definition

Disorder characterized by chronic urti-caria, in association with recurrent fever,bone pain, and IgM monoclonal gammopa-thy

Pathogenesis

May be related to deposition of the IgMparaprotein, leading to immune complexdeposition and complement activation


Chronic urticaria; individual episode usu-ally resolves within few hours; fevers per-sist for up to 24–48 hours; relapsingarthralgias; bone pain involving tibia,femur, ileum, and vertebral column; myal-gias; fatigue; weight loss


Urticarial vasculitis; lupus erythematosus;adult Still disease; Waldenström mac-roglobulinemia; chronic hepatitis B infec-tion

Therapy

Acute disease flare: prednisone


Scleredema adultorum of Buschke 519

S

References

Lipsker D, Veran Y, Grunenberger F, Cribier B, Heid E, Grosshans E (2001) The Schnitzler syn-drome. Four new cases and review of the litera-ture. Medicine 80(1):37–44

Schnitzler’s syndrome

�

Schnitzler syndrome

Schönlein-Henoch purpura

�

Henoch-Schönlein purpura

Schwannoma

�

Granular cell tumor

Schweninger and Buzzi, idiopathic anetoderma of

� Anetoderma

Scleredema

Synonym(s)Scleredema adultorum; scleredema adul-torum of Buschke; scleredema diabeti-corum; scleredema diabeticorum of Bus-chke

DefinitionDisorder characterized by nonpitting, indu-rated plaques and histological evidence ofdermal mucin deposition

PathogenesisIncreased procollagen synthesis

Clinical manifestationGroup 1 subtype: precedes febrile illness,particularly upper respiratory tract strepto-coccal infection; usually clears in 6 monthsto 2 yearsGroup 2 subtype: no prior history of febrileillness; insidious onset of skin lesions; atrisk of developing paraproteinemias,including multiple myelomaGroup 3 subtype: prior history of diabetesmellitus, usually adult onset and insulindependent, unremitting course; ill-defined,woody, nonpitting, indurated plaques; ery-thema, hyperpigmentation, and/or a peaud’orange appearance; usually located onface, neck, trunk, or upper extremities

Differential diagnosisScleroderma; lichen myxedema; general-ized myxedema; sclerema neonatorum;amyloidosis; cellulitis; erysipelas


ReferencesTate BJ, Kelly JW, Rotstein H (1996) Scleredema of

Buschke: a report of seven cases. Australasian Journal of Dermatology 37(3):139–142

Scleredema adultorum

� Scleredema

Scleredema adultorum of Buschke

� Scleredema


520 Scleredema diabeticorum

Scleredema diabeticorum

� Scleredema

Scleredema diabeticorum of Buschke

� Scleredema

Scleredema of Buschke

� Scleredema

Sclerema

� Sclerema neonatorum

Sclerema neonatorum

Synonym(s)Sclerema

DefinitionDisorder of the subcutaneous fat in debili-tated neonates, resulting in generalized sub-cutaneous plaques

PathogenesisPrematurity, hypothermia, shock, and met-abolic abnormalities increases saturated-to-unsaturated fatty acid ratio, possibly as aresult of enzymatic alteration, allowing pre-cipitation of fatty acid crystals withinlipocytes; occurs with prematurity, pneu-monia, septicemia, respiratory distress syn-

drome, congenital heart defects, gastroen-teritis, and intestinal obstruction

Clinical manifestationFirm, violaceous subcutaneous plaquesappearing suddenly, first on thighs and but-tocks and then spreading; may affect allparts of the body except palms, soles, andgenitalia; temperature instability; restrictedrespiration; difficulty in feeding; decreasedspontaneous movement

Differential diagnosisScleredema; scleroderma; subcutaneous fatnecrosis of newborn; neonatal cold injury

TherapyNo specific therapy for skin disorder; insti-tution of therapy specific for the underly-ing disease

ReferencesFretzin DF, Arias AM (1987) Sclerema neonato-

rum and subcutaneous fat necrosis of the new-born. Pediatric Dermatology 4(2):112–122

Scleroderma

Synonym(s)None

DefinitionGroup of disorders characterized by skinthickening secondary to increased dermalcollagen production


ReferencesHaustein UF (2002) Systemic sclerosis-scleroder-

ma. Dermatology Online Journal 8(1):3

Sclerodermoid fasciitis

� Eosinophilia-myalgia syndrome


Scrub typhus 521

S

Sclerodermoid myalgia

� Eosinophilia-myalgia syndrome

Scleroma

� Rhinoscleroma

Scleromyxedema


Scleromyxedema-like illness of hemodialysis

� Nephrogenic fibrosing dermopathy

Scleromyxedema-like illness of renal disease

� Nephrogenic fibrosing dermopathy

Sclerosing angioma

� Dermatofibroma

Sclerosing epithelial hamartoma


Sclerosing hemangioma

� Dermatofibroma

Sclerosing periphlebitis of the lateral chest wall

� Mondor’s disease

Scrofuloderma


Scrotal tongue

� Lingua plicata

Scrub typhus

Synonym(s)Tsutsugamushi disease; tsutsugamushifever

DefinitionAcute, febrile, infectious illness caused byRickettsia tsutsugamushi, characterized byrash and systemic signs and symptoms

PathogenesisCaused by Rickettsia tsutsugamushi (Rick-ettsia orientalis), acquired when infectedchigger bites and inoculates pathogens

Clinical manifestationHigh, severe headache, myalgia; ocularpain; wet cough; malaise; injected conjunc-tiva; eruption begins as a red, indurated


522 Scurvy

papule that eventually enlarges to 8–12 mm,vesiculates, and ruptures, developing necro-sis; 5–8 days later, onset of centrifugal-spreading macular eruption on trunk,sometimes becoming papular

Differential diagnosisTularemia; leptospirosis; typhoid fever;other rickettsial infections; viral exanthem;dengue fever

TherapyDoxycycline�; chloramphenicol – 500 mgPO 4 times daily for 7–14 days

ReferencesBaxter JD (1996) The typhus group. Clinics in

Dermatology 14(3):271–278

Scurvy

Synonym(s)Vitamin C deficiency syndrome

DefinitionVitamin C deficiency disease manifested bygingival lesions, hemorrhage, arthralgia,loss of appetite, and listlessness

PathogenesisVitamin C deficiency, after at least 3 monthsof severe or total lack of vitamin C, result-ing in defective collagen synthesis anddefective folic acid and iron utilization

Clinical manifestationPerifollicular hyperkeratotic papules, sur-rounded by a hemorrhagic halo; hairs aretwisted like corkscrews and may be frag-mented; submucosal gingival bleeding; sub-periosteal hemorrhage causes painful bonesof the legs and elsewhere; arthralgia; ano-rexia; listlessness; conjunctival hemor-rhage; poor wound healing

Differential diagnosisVasculitis; physical abuse; coagulationabnormalities with leukemia; platelet

abnormalities; deep vein thrombosis;thrombophlebitis

TherapyAscorbic acid 800–1000 mg per day PO forat least 1 week, then 400 mg per day untilrecovery complete

ReferencesHirschmann JV, Raugi GJ (1999) Adult scurvy.


Scutula

DefinitionDense masses of mycelium and epithelialdebris forming yellowish, cup-shapedcrusts, seen in the favus form of tinea capi-tis

ReferencesQianggiang Z, Limo Q, Jiajun W, Li L (2002) Re-

port of two cases of tinea infection with scutu-la-like lesions caused by Microsporum gypseum. International Journal of Dermatolo-gy 41(6):372–373

Sea boot foot

� Immersion foot

Sea lice

� Seabather’s eruption

Seabather’s eruption

Synonym(s)Sea lice


Sebaceous carcinoma 523

S

DefinitionPruritic, papular eruption occurring under-neath the swimsuit after extended expo-sure to seawater

PathogenesisHypersensitivity reaction to larval form ofthe thimble jellyfish, Linuche unguiculata;factors promoting larval venom discharge:wearing of bathing suits for prolonged peri-ods following swimming, exposure to freshwater through showering, and mechanicalstimulation

Clinical manifestationOnset a few hours after ocean bathing; pru-ritic papules in a bathing suit distributionpattern; occurence in axilla and on chest inmen with significant chest hair

Differential diagnosisCercarial dermatitis; insect bite reaction;scabies; folliculitis; jellyfish sting; urticaria

TherapyCorticosteroids, topical, high potency; anti-histamines, first generation, for sedation

ReferencesWong DE, Meinking TL, Rosen LB (1994) Seabath-

er's eruption. Clinical, histologic, and immu-nologic features. Journal of the American Academy of Dermatology 30(3): 399–406

Sebaceoma

� Sebaceous epithelioma

Sebaceous adenoma

Synonym(s)None

DefinitionBenign epithelial neoplasm composed ofsebaceous gland-like structures with seba-ceous differentiation

PathogenesisGenetic predisposition exists in some casesof Muir-Torre syndrome

Clinical manifestationYellow, tan, or pink, speckled, smooth-sur-faced, well-circumscribed papule or nod-ule, sometimes with central umbilication,located on face, scalp, or neck

Differential diagnosisBasal cell carcinoma; sebaceous carcinoma;sebaceous gland hyperplasia; nevus seba-ceous; xanthoma; xanthelasma; molluscumcontagiosum; other adnexal neoplasms


ReferencesIezzi G, Rubini C, Fioroni M, Piattelli A (2002) Se-

baceous adenoma of the cheek. Oral Oncology 38(1):111–113

Sebaceous carcinoma

Synonym(s)Sebaceous gland carcinoma

DefinitionAggressive, malignant, cutaneous tumor,arising from sebaceous glands in the skin


Clinical manifestationFirm, slowly enlarging, flesh-coloredpapule, located on upper eyelid, scalp, orneck; metastatic potential


524 Sebaceous cyst

Differential diagnosisKeratoconjunctivitis; blepharoconjunctivi-tis; chalazion; squamous cell carcinoma;basal cell carcinoma; Merkel cell carci-noma; pyogenic granuloma; melanoma;metastasis; benign adnexal tumor; sar-coidosis, ocular pemphigoid

TherapyMohs micrographic surgery�

ReferencesSnow SN, Larson PO, Lucarelli MJ, Lemke BN,

Madjar DD (2002) Sebaceous carcinoma of the eyelids treated by mohs micrographic surgery: report of nine cases with review of the litera-ture. Dermatologic Surgery 28(7):623–631

Sebaceous cyst

� Epidermoid cyst

Sebaceous epithelioma

Synonym(s)Sebaceoma

DefinitionBenign cutaneous tumor composed of lessthan 50 % of cells having sebaceous differ-entiation


Clinical manifestationFirm, flesh-colored or yellowish, smooth,sessile, or pedunculated papule on face,scalp, or eyelid; older lesions may formplaque and ulcerate

Differential diagnosisSebaceous carcinoma; squamous cell carci-noma; basal cell carcinoma; Merkel cell car-

cinoma; chalazion; pyogenic granuloma;melanoma; metastasis; sarcoidosis


ReferencesBrown MD (2000) Recognition and management

of unusual cutaneous tumors. Dermatologic Clinics 18(3):543–552

Sebaceous gland carcinoma

� Sebaceous carcinoma


� Sebaceous hyperplasia

Sebaceous hyperplasia

Synonym(s)Sebaceous gland hyperplasia; senile seba-ceous adenoma; senile sebaceous hyperpla-sia

DefinitionHamartomatous enlargement of facial seba-ceous glands, characterized by yellowpapules with central dell

PathogenesisOccurs commonly in organ transplantrecipients, suggesting immune mecha-nisms in some cases

Clinical manifestationWell-demarcated, yellow-to-flesh-colored,delled papules, most commonly on fore-head and cheeks


Seborrheic dermatitis 525

S

Differential diagnosisSebaceous carcinoma; melanocytic nevus;sebaceous adenoma; sebaceous epitheli-oma; squamous cell carcinoma; basal cellcarcinoma; sarcoidosis; colloid milium;fibrous papule; granuloma annulare; lipoidproteinosis; milium; molluscum contagio-sum; syringoma; trichoepithelioma; xan-thoma; xanthelasma

TherapyLight electrodesiccation; liquid nitrogencryotherapy; laser ablation; shave removal;isotretinoin for multiple lesions

Referencesde Berker DA, Taylor AE, Quinn AG (1996) Seba-

ceous hyperplasia in organ transplant recipi-ents: shared aspects of hyperplastic and dysplastic processes? Journal of the American Academy of Dermatology 35(5 Pt 1): 696–699

Sebocystomatosis

� Steatocystoma multiplex

Seborrhea


Seborrhea capitis


Seborrheic blepharitis


Seborrheic dermatitis

Synonym(s)Seborrhea; dandruff; seborrheic eczema;seborrhea capitis; pityriasis sicca; pityriasissimplex capitis; pityriasis oleosa; pityriasiscorporis; seborrheic blepharitis

DefinitionInflammatory dermatosis in areas with highsebum flow and accumulation, such as thescalp, face, intertriginous areas, and chest

PathogenesisAbnormal immune response to a normalconstituent of the skin flora, Pityrosporumovale

Clinical manifestationScalp: appearance varies from mild, patchyscaling to widespread, thick, adherentcrustsFace: central facial erythema and scale,most prominent in skin foldsEyelids: poorly defined, scaly, reddish-brown plaquesPresternal or interscapular area: poorlydefined, red-brown, scaly papules andplaquesIntertriginous areas: fairly sharply demar-cated, red, scaly plaques

Differential diagnosisTinea capitis; atopic dermatitis; psoriasis;intertrigo; contact dermatitis; candidiasis;

Seborrheic dermatitis. Scaly plaques on the central face


526 Seborrheic eczema

diaper dermatitis; pityriasis rosea; pityria-sis lichenoides chronica; lupus erythemato-sus; rosacea; Darier disease; Hailey-Haileydisease; Grover’s disease; pemphigusfoliaceus; xerotic eczema; chronic granulo-matous disease; exfoliative erythroderma;infectious eczematoid dermatitis; Letterer-Siwe disease; staphylococcal blepharitis;tinea amiantacea; vitamin B and/or zincdeficiency; glucagonoma syndrome

TherapyAnti-seborrheic shampoo, used daily; corti-costeroids, topical, low potency for face;corticosteroids, topical, mid potency fortrunk; azole antifungal agents seborrheicblepharitis: scrubbing of eyelids daily withbaby shampoo diluted 1 : 1 with water

ReferencesFaergemann J (2000) Management of seborrheic

dermatitis and pityriasis versicolor. American Journal of Clinical Dermatology 1(2):75–80

Seborrheic eczema


Seborrheic keratosis

Synonym(s)Seborrheic wart; senile wart; basal cell pap-illoma

DefinitionBenign tumor composed of epidermalkeratinocytes

PathogenesisHereditary component; sunlight may be afactor in some cases

Clinical manifestationNon-inflamed, single or multiple, sharplydefined, flesh-colored, light brown, gray,blue, or black, flat papules with a velvety or

finely verrucous surface; edges raised offskin surface, giving lesion a “stuck-on”appearanceDermatosis papulosa nigra variant: small,pedunculated, heavily pigmented papule,with minimal keratotic element, on faceStucco keratosis variant: superficial, gray-to-light-brown, flat, keratotic papules onthe dorsa of the feet, ankles, hands, andforearmsMelanoacanthoma variant: deeply pig-mented keratotic plaque with histologic evi-dence of proliferation or activation of den-dritic melanocytes

Differential diagnosisMelanocytic nevus; melanoma; acrochor-don; actinic keratosis; basal cell carcinoma;squamous cell carcinoma; psoriasis; pem-phigus foliaceus; wart

TherapyElectrodesiccation and curettage; liquidnitrogen cryotherapy; shave removal; ellip-tical excision



Seborrheic wart


Secret antiperspirant


Sedge pool itch



Self-healing squamous cell carcinoma 527

S

Segmental hyalinizing vasculitis


Seidlmayer syndrome


Seip syndrome


Selective serotonin reuptake inhibitor (SSRI)

Trade name(s)Generic names in parentheses:Celexa (citalopram); Zoloft (sertraline);Prozac (fluoxetine); Paxil (paroxetine);Luvox (fluvoxamine)

Generic availableYes – fluoxetine; fluvoxamine

Drug classSelective serotonin reuptake inhibitor

Mechanism of actionInhibits serotonin reuptake at the presynap-tic terminal

Dosage formCelexa: 20 mg, 40 mg tabletZoloft: 25 mg, 50 mg, 100 mg tablet

Prozac: 10 mg, 20 mg tablet, 20 mg per 5 mlliquidPaxil: 20 mg, 30 mg tabletLuvox: 25 mg, 50 mg, 100 mg tablet


Common side effectsCutaneous: skin eruptionGastrointestinal: anorexia, hyperexiaGenitourinary: sexual dysfunctionNeurologic: insomnia, sedation, headache

Serious side effectsNeurologic: serotonin syndrome

Drug interactionsBuspirone; cimetidine; ergot alkaloids; eth-anol; anti-psychotics, both typical andatypical; lithium; MAO inhibitors; metopro-lol; phenytoin; quinidine; tricyclics; warfa-rin

Contraindications/precautionsHypersensitivity to drug class or compo-nent; MAO inhibitors within 14 days; avoidrapid withdrawal

ReferencesGupta MA, Guptat AK (2001) The use of antide-

pressant drugs in dermatology. Journal of the European Academy of Dermatology & Venere-ology 15(6):512–518

Self-healing epithelioma

� Keratoacanthoma

Self-healing squamous cell carcinoma

� Keratoacanthoma


528 Self-limiting acroderamatitis enteropathica

Self-limiting acroderamatitis enteropathica


Senile comedones


Senile depigmented spots


Senile elastosis


Senile hemangioma of the lips

� Venous lake

Senile keratosis

� Actinic keratosis

Selective serotonin reuptake inhibitor (SSRI). Dermatologic indications and dosage



Celexa: 20–40 mg PO once daily; Zoloft: 50–100 mg PO once daily; Prozac 10–60 mg PO once daily; Paxil 20–40 mg PO once daily; Luvox 25–100 mg PO at bedtime

Celexa: safety and effectiveness not established; Zoloft: 25 mg PO once daily (6–12 years old); Prozac 5–20 mg PO once daily; Paxil 10–30 mg PO once daily (> 8 years old); Luvox 25–50 mg PO at bedtime

Obsessive-compulsive disorders

Celexa: 20–40 mg PO once daily; Zoloft: 50–100 mg PO once daily; Prozac 10–60 mg PO once daily; Paxil 20–40 mg PO once daily; Luvox 25–100 mg PO at bedtime


Prurigo nodularis Celexa: 20–40 mg PO once daily; Zoloft: 50–100 mg PO once daily; Prozac 10–60 mg PO once daily; Paxil 20–40 mg PO once daily; Luvox 25–100 mg PO at bedtime


Trichotillomania Celexa: 20–40 mg PO once daily; Zoloft: 50–100 mg PO once daily; Prozac 10–60 mg PO once daily; Paxil 20–40 mg PO once daily; Luvox 25–100 mg PO at bedtime



Seven-year itch 529

S

Senile sebaceous adenoma


Senile sebaceous hyperplasia


Senile wart


Sertraline

� Selective serotonin reuptake inhibi-tor (SSRI)

Serum sickness

Synonym(s)None

DefinitionSelf-limited immune complex diseasecaused by exposure to foreign proteins orhaptens

PathogenesisWith slight antigen excess, intermediate-sized immune complexes deposit in smallvessels and activate complement; increasedadhesion molecule expression in endothe-

lial cells causes cytokine release and vascu-lar injury

Clinical manifestationUrticarial, morbilliform, or scarlatiniformeruption; palpable purpura; erythema mul-tiforme; facial edema; pruritus and ery-thema at injection site; symmetrical arthri-tis, usually in metacarpophalangeal andknee joints; myalgias; lymphadenopathy;splenomegaly; neurologic complications,including headache, optic neuritis; cranialnerves palsies, Guillain-Barré syndrome;gastrointestinal complaints, includingabdominal pain, nausea, vomiting,diarrhea; clinical recovery after 7–28 days

Differential diagnosisUrticaria; cryoglobulinemia; hepatitis;mononucleosis; hypersensitivity vasculitis;lupus erythematosus; Henoch-Schönleinpurpura; Still disease

TherapyAntihistamines, first generation; pred-nisone for patients with multisysteminvolvement and significant symptomatol-ogy

ReferencesRoujeau JC, Stern RS (1994) Severe adverse cuta-

neous reactions to drugs. New England Journal of Medicine 331(19):1272–1285

Seven-day fever

� Leptospirosis

Seven-year itch

� Scabies


530 Sézary's syndrome

Sézary's syndrome


Shank fever

� Trench fever

Sharp syndrome

� Mixed connective tissue disease

Sharp’s syndrome

� Mixed connective tissue disease

Shaving bumps


Sheep-pox

� Orf

Shin spots


Shinbone fever

� Trench fever

Shingles

� Herpes zoster

Short anagen syndrome


Sicca syndrome


Siemerling-Creutzfeldt syndrome


Sign of Leser-Trelat

DefinitionAbrupt appearance and growth of multipleseborrheic keratoses, caused by an underly-ing malignancy

ReferencesSchwartz RA (1996) Sign of Leser-Trelat. Journal

of the American Academy of Dermatology 35(1):88–95


Skeeter's syndrome 531

S

Silt itch


Sipple syndrome


Sitosterolemia

� Phytosterolemia

Sixth disease

� Roseola

Sjögren syndrome

Synonym(s)Sicca syndrome; Sjögren’s syndrome;Gougerot-Houwer-Sjögren syndrome;keratoconjunctivitis sicca

DefinitionChronic disorder characterized by kerato-conjunctivitis sicca and xerostomia

PathogenesisAutoimmune dysregulation, particularlypolyclonal B lymphocyte hyperreactivity;genetic susceptibility; abnormality in cellu-lar apoptosis

Clinical manifestationGlandular symptoms: dry eye syndrome,characterized by dryness of cornea andconjunctiva; dry mouth; dry lips; red,smooth dry tongue; dental caries; recurrent

oral candidiasis; recurrent salivary glandswelling; nasal dryness with recurrentinfections, hoarseness, and aphonia;atrophic changes in the vulva and vagina,resulting in pruritus and vaginitis; anal andrectal mucosal drynessSkin symptoms: xerosis; decreased sweat-ing; dry, sparse hair; annular, red, scalyplaques, especially on face and neck; cuta-neous vasculitisPrimary variant: no associated connectivetissue or autoimmune disease; extraglandu-lar involvement: lung involvement, nervoussystem dysfunction, renal involvement,Raynaud phenomenon, and lymphoprolif-erative disordersSecondary variant: associated connectivetissue or autoimmune disease; milder dis-ease with fewer systemic manifestations

Differential diagnosisHIV infection; drug reaction; lupus ery-thematosus; amyloidosis; environmentaldryness

TherapyDry eyes: Artificial tears (e.g., methylcellu-lose, 1 % hyaluronic acid solution, alcoholsolutions) applied 4–6 times dailyDry mouth: frequent small drinks andmouthwashes; artificial saliva; stimulationof salivary secretion with sweets, etc.

ReferencesManoussakis MN, Moutsopoulos HM (2001)

Sjogren's syndrome: current concepts. Advanc-es in Internal Medicine 47:191–217

Sjögren’s syndrome


Skeeter's syndrome



532 Skin tag

Skin tag

� Acrochordon

Skin writing

� Dermatographism

Slapped-cheek disease


Sleeping sickness

� African trypanosomiasis

Small cell carcinoma of the skin


Small plaque parapsoriasis

Synonym(s)Benign parapsoriasis; digitate dermatitis;digitate dermatosis; chronic superficial der-matitis; guttate parapsoriasis; Brocq’s dis-ease

DefinitionChronic, benign, cutaneous disease, charac-terized by scaly plaques resembling psoria-sis

PathogenesisMost likely represents a reactive process ofpredominantly CD4+ T cells

Clinical manifestationWell-circumscribed, slightly scaly, lightsalmon-colored papules or plaques scat-tered over the trunk and extremitiesDigitate pattern: palisading, elongated fin-gerlike plaques following a dermatomalpattern, most prominently on the flank;active lesions for months to several years;usually resolves spontaneously

Differential diagnosisPsoriasis; dermatophytosis; lupus ery-thematosus; lichen planus; pityriasis rosea;syphilis; seborrheic dermatitis; mycosisfungoides; xerosis; nummular dermatitis

TherapyCorticosteroids, topical, high potency; UVBphototherapy; photochemotherapy

ReferencesLambert WC, Everett MA (1981) The nosology of

parapsoriasis. Journal of the American Acade-my of Dermatology 5(4):373–395

Small vessel vasculitis


Smallpox

� Variola

Smoker's comedones



South African porphyria 533

S

Sneddon-Wilkinson disease

� Subcorneal pustular dermatosis

Sodoku

� Rat-bite fever

Soft chancre

� Chancroid

Soft wart

� Acrochordon

Solar cheilitis


Solar comedones


Solar elastosis


Solar keratosis

� Actinic keratosis

Solar urticaria

� Urticaria

Solid cystic hidradenoma


Solitary lichen planus


Solitary lichen planus-like keratosis


Sorbsan

� Alginates

South African porphyria



534 South African tick typhus

South African tick typhus

� Boutonneuse Fever


Synonym(s)Paracoccidioidomycosis; Lutz mycosis;Brazilian blastomycosis

DefinitionSystemic mycotic infection, endemic tocountries in Central America and SouthAmerica, caused by the fungus Paracoccidi-oides brasiliensis

PathogenesisCaused by thermally dimorphic fungus,Paracoccidioides brasiliensis; acquired byinhalation of conidia fungus that trans-forms into yeast cells within alveolar mac-rophages; fungus may disseminate, causinggranulomatous disease in multiple organs;alcohol and tobacco use associated withdissemination

Clinical manifestationAdult chronic form:Mucous membranes: slowly progressive,painful papules or plaques ulcerate in oral,nasal, pharyngeal, and laryngeal tissue; gin-gival lesions cause loss of teeth; conjunctivi-tis and ulcerative lesions of the perianalareaSkin: occurs most commonly on the face;may have nodules, ulcerations or papillo-matous lesions; most often arises fromdirect extension of mucous membranelesions; hematogenous spread causes widelyscattered subcutaneous abscesses; lymphnodes: extensive hypertrophic, painful lym-phadenopathy with visceral and subcutane-ous nodes; cervical nodes commonly

affected; suppuration causes sinus tracts orskin ulcersRespiratory: lung involvement in 70-80% ofpatients and often the only organ systeminvolved; frequently resembles tuberculo-sis, with chronic dyspnea, cough, and spu-tum productionOther systemic problems: hepatosplenome-galy, adrenal insufficiency meningitis,intestinal ulcerations, and osteomyelitisJuvenile subacute form:Mucous membranes: rare mucosal ulcera-tionsSkin: acneiform eruption or subcutaneousabscesses; scrofuloderma as a result oflymph node suppurationLymph nodes: prominent lymphadenopa-thy with suppuration; mesenteric adenopa-thy may produce bowel obstructionRespiratory: occasional pneumoniaOther problems: cachexia, hepatosplenome-galy, adrenal insufficiency, osteomyelitis,gastrointestinal problems

Differential diagnosisActinomycosis; coccidioidomycosis; leish-maniasis; sporotrichosis; syphilis; tubercu-losis; histoplasmosis; North American blas-tomycosis; Wegener’s granulomatosis; oralcarcinoma; drug eruption; lymphoma;leukemia

TherapyTrimethoprim and sulfamethoxazole�;ketoconazole; itraconazole; for severe dis-ease: amphotericin B – 0.7–1 mg per kg IVdaily for 4–8 weeks, followed by trimetho-prim and sulfamethoxazole for 2–3 years



South American pemphigus

� Fogo selvagem


Spider veins 535

S

South American trypanosomiasis

� American trypanosomiasis

Spanish toxic oil syndrome

� Toxic oil syndrome

Speckled lentiginous nevus

� Nevus spilus

Speckled nevus spilus

� Nevus spilus

Spectacle frame granuloma

� Acanthoma fissuratum

Sphingomyelin-cholesterol lipidoses


Spider angioma

Synonym(s)Spider nevus; nevus araneus; vascular spi-der

DefinitionVascular lesion consisting of central arteri-ole with radiating, thin-walled vessels

PathogenesisDilation of pre-existing vessels; sometimesoccurs in patients with cirrhosis or otherhepatic abnormalities; elevated blood estro-gen a common characteristic

Clinical manifestationRed macule or papule surrounded by sev-eral distinct radiating vessels, occurringmost commonly on face, below eyes, andover cheekbones; central pressure causeslesion to blanch

Differential diagnosisTelangiectatic mat; spider telangiectasia;insect bite; cherry angioma

TherapyDestruction by electrodesiccation or laservaporization

ReferencesRequena L, Sangueza OP (1997) Cutaneous vascu-

lar anomalies. Part I. Hamartomas, malforma-tions, and dilation of preexisting vessels. Journal of the American Academy of Dermatol-ogy 37(4):523–549

Spider nevus

� Spider angioma

Spider veins



536 Spiegler-Fendt, pseudolymphoma of

Spiegler-Fendt, pseudolymphoma of

� Pseudolymphoma

Spiegler-Fendt sarcoid

� Pseudolymphoma

Spiradenoma

Synonym(s)Eccrine spiradenoma

DefinitionBenign tumor of sweat gland origin, pre-senting as a solitary gray-pink papule

PathogenesisUnclear whether tumor arises from apo-crine or eccrine epithelium

Clinical manifestationSolitary firm, gray-pink papule, usuallyarising in the head and neck region ortrunk; occasional pain and tenderness

Differential diagnosisCylindroma; basal cell carcinoma; trichoep-ithelioma; eccrine poroma; angiofibroma;milium


ReferencesMichal M (1996) Spiradenoma associated with

apocrine adenoma component. Pathology, Re-search & Practice 192(11):1135–1139

Spirillary fever

� Rat-bite fever

Spironolactone

Trade name(s)Aldactone


Drug classDiuretic; anti-androgen

Mechanism of actionAndrogen receptor antagonist on sebaceusglands; inhibits androgen synthesis

Dosage form25 mg, 50 mg, 100 mg tablet


Spironolactone. Dermatologic indications and dosage


Acne vulgaris 25–100 mg PO twice daily Not indicated


25–100 mg PO twice daily Not indicated


25–100 mg PO twice daily Not indicated

Hirsutism 25–100 mg PO twice daily Not indicated


Sporotrichosis 537

S

Common side effectsDermatologic: skin eruptionGastrointestinal: dyspepsiaNeurologic: sedation, headacheGenitourinary: sexual dysfunction, dys-menorrhea

Serious side effectsDermatologic: anaphylaxisBone marrow: marrow suppression

Drug interactionsACE inhibitors; cyclosporine; non-steroidalanti-inflammatory agents; COX-2 inhibi-tors; potassium salts; tacrolimus

Contraindications/precautionsHypersensitivity to drug class or compo-nent; renal insufficiency; hyperkalemia;caution in patients with liver dysfunction

ReferencesThiboutot D (2001) Hormones and acne: patho-

physiology, clinical evaluation, and therapies. Seminars in Cutaneous Medicine & Surgery 20(3):144–53

Splash rash


Sporotrichosis

Synonym(s)Schenck's disease; Beurmann's disease; rosegardener's disease; peat moss disease

DefinitionSubcutaneous or systemic fungal infectioncaused by soil pathogen, Sporothrixschenckii

PathogenesisCaused by Sporothrix schenckii, dimorphicfungus commonly found on vegetative mat-ter, particularly in humid climates; may

gain entry through puncture wound,spreading via lymphatic vessels

Clinical manifestationHistory of prick injury at site of infection,within 3 weeks of onset of signs and symp-tomsLymphocutaneous variant: subcutaneousnodule developing at site of inoculation andsometimes ulcerating after central abscessformation; satellite lesions along associatedlymphatic chain with lymphadenopathyFixed cutaneous variant: scaly, acneform,verrucous, or ulcerative nodule remaininglocalized to site of inoculationDisseminated variant: multiple organinvolvement causing pyelonephritis, orchi-tis, mastitis, arthritis, synovitis, meningi-tis, osseous infection, or (rarely) pulmo-nary disease

Differential diagnosisAtypical mycobacterial infection; nocardio-sis; North American blastomycosis; SouthAmerican blastomycosis; leishmaniasis;bacterial pyoderma; anthrax; cutaneoustuberculosis; tularemia; foreign body gran-uloma; herpes zoster

TherapyLymphocutaneous variant: itraconazole�;saturated solution of potassium iodide:300–500 mg PO 3 times daily for 4–8 weeks Disseminated variant: amphotericin B –3 mg per kg per day IV until significantclinical response; itraconazole

Sporotrichosis. Linear, scaly, red papules, one of which is ulcerated


538 Sportsman’s toe

ReferencesMorris-Jones R (2002) Sporotrichosis. Clinical &

Experimental Dermatology 27(6):427–431

Sportsman’s toe

� Tennis toe

Spotted fever


Spotted leg syndrome


Spun glass hair



Synonym(s)Epidermoid carcinoma; prickle cell carci-noma

DefinitionMalignant tumor of keratinocytes, mostoften arising in chronically sun-exposedskin

PathogenesisRelated closely to chronic sun exposure;other risk factors: immunosuppression, faircomplexion, history of ionizing radiation orphotochemotherapy, abnormal DNA repairmechanisms, infection with certain human

papillomavirus virus subtypes, and localsites of chronic inflammation

Clinical manifestationElevated, firm, pink to flesh-colored, kera-totic papule or plaque with or without over-lying cutaneous horn or ulceration, oftenarising from pre-existing actinic keratosis;lip lesion: most commonly on vermillionborder of lower lip; shiny, ulcerated papuleor nodule

Differential diagnosisActinic keratosis; basal cell carcinoma;benign adnexal neoplasm; melanoma; Mer-kel cell carcinoma; atypical fibroxanthoma;seborrheic keratosis; wart; pyogenic granu-loma; proliferating trichilemmal cyst; gran-ular cell tumor; granulomatous diseasessuch as tuberculosis, leishmaniasis, coccidi-oidomycosis, North American blastomyco-sis, syphilis, and bromoderma

TherapySurgical excision; destruction by electro-desiccation and curettage or liquid nitro-gen cryotherapy; superficial orthovoltageradiation therapy; large tumors, lesions inanatomically sensitive areas, or recurrenttumors – Mohs micrographic surgery orother form of microscopically controlledexcision

ReferencesAn KP, Ratner D (2001) Surgical management of

cutaneous malignancies. Clinics in Dermatolo-gy 19(3):305–320

Squamous cell carcinoma. Scaly red papule with central erosion


Stasis dermatitis 539

S

Squamous cell carcinoma in-situ


SSSS

� Staphylococcal scalded skin syn-drome

Staphylococcal scalded skin syndrome

Synonym(s)SSSS; scalded skin syndrome; pemphigusneonatorum

DefinitionToxin-mediated disease of young children,characterized by acute generalized skinexfoliation

PathogenesisCaused by toxigenic strains of Staphylococ-cus aureus, usually belonging to phagegroup 2 (types 3A, 3B, 3C, 55, or 71); twoexotoxins (ETs), epidermolytic toxin A (ET-A) and epidermolytic toxin B (ET-B),responsible for the pathologic changes andblistering produced by disruption of epi-dermal granular cell layer

Clinical manifestationOriginal focus of infection may be purulentconjunctivitis, otitis media, or nasopharyn-geal infection; fever; irritability; general-ized, faint, orange-red, macular erythemawith cutaneous tenderness and periorificialand flexural accentuation; early positiveNikolsky sign; within 24–48 hours, rashprogresses to generalized, superficial blis-tering eruption, with tissue paper-like sur-

face wrinkling, followed by large, flaccidbullae in axillae, groin, and around thebody orifices, sparing mucous membranes;after epidermal sloughing, moist erythema-tous base present; healing usually completewithin 5–7 days

Differential diagnosisToxic shock syndrome; Kawasaki disease;scarlet fever; erythema multiforme; childabuse

TherapyDicloxacillin�

ReferencesVeien NK (1998) The clinician's choice of antibiot-

ics in the treatment of bacterial skin infection. British Journal of Dermatology 139 Suppl 53:30–36

Staphylococcal toxic shock syndrome

� Toxic shock syndrome

Stasis dermatitis

Synonym(s)Venous eczema

DefinitionInflammatory disease of the lower extremi-ties, characterized by eczematous changesin the context of chronic pedal edema

PathogenesisResult of venous insufficiency; disturbedfunction of the deep venous plexus valvu-lar system with backflow of blood from thedeep to the superficial venous system, pro-ducing venous hypertension; possiblyrelated to leukocyte sequestration in micro-circulation, with increased contact of leuko-cytes with the capillary endothelium and


540 Steatoblepharon

release of inflammatory mediators; leuko-cyte sludging may block dermal capillaries,leading to tissue ischemia

Clinical manifestationErythematous, scaling, eroded plaques oflower extremity; medial ankle most fre-quently and severely involved; acute flareswith exudative, weeping plaques; long-standing lesions with lichenification andhyperpigmentation; skin induration some-times progresses to significant scarringlipodermatosclerosis and violaceousplaques and nodules on the legs and dorsalfeet (acroangiodermatitis)

Differential diagnosisContact dermatitis; cellulitis; Kaposi’s sar-coma; atopic dermatitis; xerotic eczema;necrobiosis lipoidica; nummular eczema;dermatophytosis; benign pigmented pur-pura; pretibial myxedema

TherapyCorticosteroids, topical, mid potency�;compression therapy with Unna boot dress-ings, controlled gradient compressiondevice or compression stockings; pred-nisone for severe acute flares

ReferencesWeingarten MS (2001) State-of-the-art treatment

of chronic venous disease. Clinical Infectious Diseases 32(6):949–954

Steatoblepharon

� Dermatochalasis

Steatocystoma


Steatocystoma multiplex

Synonym(s)Steatocystoma; sebocystomatosis

DefinitionHeritable disorder of pilosebaceous unit,characterized by multiple sebum-contain-ing cysts

PathogenesisAutosomal dominant trait; hamartomatousformation of abortive hair follicles at sitewhere sebaceous glands attach; associatedwith vellus hair cysts and trichostasisspinulosa, and sometimes existing on spec-trum with these entities

Clinical manifestationAsymptomatic, smooth, flesh-to-yellow-colored papules; occasional rupture into thedermis producing inflammation with scar-ring; concentrated over upper torso, proxi-mal extremities; contents of lesion odor-less, creamy or oily fluid; non-hereditaryvariant: solitary lesion morphologicallyidentical to multiple lesions (steatocystomasimplex)

Stasis dermatitis. Scaly, crusted, and eroded plaque on the lower extremity


Stevens-Johnson syndrome 541

S

Differential diagnosisAcne vulgaris; epidermoid cyst; trichilem-mal cyst; eruptive vellus hair cyst; milia;syringoma; Gardner syndrome

TherapyTetracycline; isotretinoin; surgical excisionof individual inflammatory lesions

ReferencesRollins T, Levin RM, Heymann WR (2000) Acral

steatocystoma multiplex. Journal of the Ameri-can Academy of Dermatology 43(2 Pt 2):396–369

Steatocystoma simplex


Steatoma

� Epidermoid cyst

Steely hair syndrome

� Menkes kinky hair syndrome

Sterile eosinophilic pustulosis


Steroid acne

� Acne medicamentosa

Steroid rosacea


Stevens-Johnson syndrome

Synonym(s)Erythema multiforme major

DefinitionSystemic hypersensitivity reaction, mainlyinvolving the skin and mucous membranes

PathogenesisCell-mediated immune response, mediatedby CD8 lymphocytes; may involve an HLA-DQw3-related, altered immune response;associated with medications, such as sul-fonamides, penicillin, or anti-convulsants,and with infections (most commonly, her-pes simplex virus infection and myco-plasma pneumonia)

Clinical manifestationErythematous papules, vesicles, bullae, andtarget-like papules, mainly on face, trunk,and mucous membranes, including oral,genital mucosa; < 35 % of body surfaceinvolved; lesions may be located on liningsof respiratory and gastrointestinal tracts;conjunctivitis with photophobia; burningsensation in eyes; hepatitis; nephritis; gas-trointestinal bleeding; pneumonia; myal-gia; arthritis; arthralgia

Differential diagnosisPemphigus vulgaris, erosive lichen planus;varicella zoster infection; Behcet's disease;Reiter's syndrome; herpes simplex virusinfection; bullous pemphigoid; toxic epider-mal necrolysis; Henoch-Schönlein pur-pura; urticaria; viral exanthem; Kawasakidisease; figurate erythema; fixed drug erup-tion; lupus erythematosus; aphthous stoma-titis


542 Stewart-Bluefarb syndrome

TherapyPrednisone

ReferencesPrendiville J (2002) Stevens-Johnson syndrome

and toxic epidermal necrolysis. Advances in Dermatology 18:151–173

Stewart-Bluefarb syndrome


Stewart-Treves syndrome

Synonym(s)Lymphangiosarcoma of Stewart-Treves

DefinitionMalignant vascular tumor arising in an areaof chronic lymphedema, particularly onupper extremity after radical mastectomy

PathogenesisOccurs in the context of chronic lymphe-dema

Clinical manifestationPurplish patch, evolving into plaque ornodule in the area of chronic lymphedema;palpable subcutaneous mass or poorly heal-ing eschar with recurrent bleeding and ooz-ing; nodules may become polypoid,develop small satellite papules and becomeconfluent; overlying epidermis sometimesulcerates, producing recurrent episodes ofbleeding and infection; high metastaticpotential

Differential diagnosisAngioendotheliomatosis; angiolymphoidhyperplasia with eosinophilia; Kaposi’s sar-coma; lymphangioma; melanoma; metasta-sis; hemangioendothelioma; hemangioperi-cytoma

TherapyRadical amputation of the limb�; radiationtherapy

ReferencesChung KC, Kim HJ, Jeffers LL (2000) Lymphangi-

osarcoma (Stewart-Treves syndrome) in post-mastectomy patients. Journal of Hand Surgery – American Volume 25(6):1163–1168

Sticker disease


Sticker's disease


Stomatitis areata migrans


Stork bite

� Salmon patch

Strawberry hemangioma


Strawberry mark



Streptococcal toxic shock-like syndrome 543

S

Strawberry patch

� Nevus flammeus

Streeter’s dysplasia


Streeter's spots


Strep toxic shock syndrome

� Streptococcal toxic shock-like syn-drome

Strep toxic shocklike syndrome


Streptobacillary fever

� Rat-bite fever

Streptococcal toxic shock-like syndrome

Synonym(s)Strep toxic shock-like syndrome; strepto-coccal TSS flesh eating disease

DefinitionAcute febrile illness, characterized by signsof localized infection, often in the skin; gen-eralized erythematous eruption accompa-nied by shock and multiple organ dysfunc-tion

PathogenesisCaused by strains of Streptococcus pyo-genes; superantigen behavior of pyrogenicexotoxin-A (SPE-A); may also producestreptococcal pyrogenic exotoxin-B (SPE-B), streptococcal pyrogenic exotoxin-C(SPE-C), streptococcal superantigen andmitogenic factor, as well as non–group-Astreptococci aureus; release of tumor necro-sis factor-α (TNF-α) and interleukin-1b (IL-1b), which mediate signs and symptoms ofdisease; predisposing factors: influenza A,soft tissue wounds, varicella, pneumonia,unidentified bacteremia, surgical site infec-tion, septic arthritis, thrombophlebitis,meningitis, pelvic infection, endophthalmi-tis; additional risk factors: HIV, diabetesmellitus, cancer, ethanol abuse, and otherchronic diseases

Clinical manifestationLocalized pain in an extremity, rapidly pro-gressing over 48–72 hoursCutaneous signs: localized edema and ery-thema; bullous and hemorrhagic cellulitis;necrotizing fasciitis or myositis; gangreneOther organ involvement: fever; hypoten-sion; cardiomyopathy; nausea; vomiting;diarrhea; rhabdomyolysis; myalgias; mus-cle tenderness and weakness; azotemia;acute renal failure; adult respiratory dis-tress syndrome; elevated serum glutamicoxaloacetic transaminase (SGOT) andserum bilirubin; thrombocytopenia; leuko-cytosis; disseminated intravascular coagu-lation; hypophosphatemia; hypocalcemia;and electrolyte imbalance

Differential diagnosisToxic shock syndrome; Stevens-Johnsonsyndrome; Kawasaki disease; staphylococ-cal scalded skin syndrome; toxic epidermalnecrolysis; drug reaction; scarlet fever;


544 Streptococcal TSS flesh eating disease

Rocky Mountain spotted fever; leptospiro-sis; gas gangrene; meningococcemia

TherapyNafcillin: 2 gm IV every 4 hours in adults;100–200 mg per kg per day divided into 4–6doses per day in childrenClindamycin: 600–900 mg IV every 8 hoursin adults; 20–40 mg per kg per day IVdivided into 3–4 doses in childrenIntravenous immunoglobulin (IVIG) 1–2 gm per kg over 2–3 days

ReferencesLevine N., Kunkel M, Thanh N; Ackerman L

(2002) Emergency department dermatology. Current Problems in Dermatology 14(6):188–220

Streptococcal TSS flesh eating disease


Stretch marks

� Striae

Striae

Synonym(s)Striae distensae; striae atrophicans; striaerubra; striae alba; stretch marks

DefinitionLinear dermal scars accompanied by epi-dermal atrophy

PathogenesisResults from stress rupture of dermal con-nective tissue framework; affects skin sub-jected to continuous and progressive

stretching; skin distension causes excessivemast cell degranulation with subsequentdamage of collagen and elastin; maydevelop more easily in skin with high pro-portion of rigid cross-linked collagen; asso-ciated with increased adrenal cortical hor-mone activity, such as in Cushing’s diseaseor with exogenous glucocorticoid therapy

Clinical manifestationFlattened, atrophic plaques with a pink hue,which enlarge in length and width andbecome violaceous; older striae are white,depressed, irregularly shaped bands withtheir long axis parallel to skin tension lines;in pregnancy, striae affect abdomen andbreasts; adolescent striae occur on outeraspects of thighs and lumbo-sacral regionin boys, and thighs, buttocks, and breasts ingirls; flexures affected with topical corticos-teroid use, especially if used under occlu-sion

Differential diagnosisLinear focal elastosis; Marfan syndrome;Cushing’s syndrome; external trauma

Therapy585-nm flashlamp pumped dye laser; tretin-oin; chemexfoliation with trichloroaceticacid

ReferencesMcDaniel DH, Ash K, Zukowski M (1996) Treat-

ment of stretch marks with the 585-nm flash-lamp-pumped pulsed dye laser. Dermatologic Surgery 22(4): 332–337

Striae. Linear, red-brown, atrophic plaques


Strongyloidosis 545

S

Striae alba

� Striae

Striae atrophicans

� Striae

Striae distensae

� Striae

Striae rubra

� Striae

Striate keratoderma

Synonym(s)Keratoderma palmoplantaris striata; striatepalmoplantar keratoderma; Brunaur-Fuhs-Siemens syndrome; palmoplantar kerato-derma areata

DefinitionDisorder characterized by linear or striatepatterns of keratoderma radiating from thepalm and extending along the fingers

PathogenesisAutosomal dominant trait; mutations ingene encoding for desmoglein 1 and desmo-plakin

Clinical manifestationLinear or striate keratotic plaques radiatingalong the digits from the palm; onset from

5–20 years of age; diffuse thickening ofpalms and soles and plaques or islands ofincreased keratin developing at pressuresites

Differential diagnosisWart; callus; focal nonepidermolytic pal-moplantar keratoderma; focal epidermo-lytic palmoplantar keratoderma; focal pal-moplantar and oral mucosa hyperkeratosis;tyrosinemia type II; punctate keratoderma;acrokeratoelastoidosis; focal acral hyperk-eratosis

TherapyAcitretin�

ReferencesHelm T, Spigel GT, McMahon J, Bergfeld WF

(1998) Striate palmoplantar keratoderma: a clinical and ultrastructural study. Cutis 61(1):18–20

Striate palmoplantar keratoderma

� Striate keratoderma

Strongyloidosis

Synonym(s)Strongylydiasis; cutaneous strongyloidia-sis; human threadworm infection;anguillulosis; Cochin China diarrhea

DefinitionParasitic infection of the organism Strongy-loides stercoralis

PathogenesisInfection acquired when infective filari-form larvae penetrate the skin during con-tact with contaminated soil; immunosup-pression a risk factor for wide dissemina-tion


546 Strongylydiasis

Clinical manifestationMild, pruritic eruption of feet, site of inocu-lation of larvae; larva currens (creepingeruption), a form of cutaneous larvamigrans specific to Strongyloides infection,and a result of autoinfection; rapidlyspreading pruritic eruption in perianalregion; with disseminated infection,expanding petechial and purpuric lesions,sometimes accompanied by pink maculesand papules; gastrointestinal findings:abdominal tenderness; distension; hyperac-tive, hypoactive, or absent bowel sounds;central nervous system infection: alteredmental status; meningismus; pulmonaryfindings: coughing; respiratory distress;wheezing

Differential diagnosisScabies; contact dermatitis; cat or doghookworm infestation; pinworm infesta-tion; bacterial pyoderma

TherapyIntestinal stage: ivermectin�; albendazole;disseminated disease: thiabendazole 1.5 gper dose PO twice daily for 2–3 days

ReferencesSchneider JH, Rogers AI (1997) Strongyloidiasis.

The protean parasitic infection. Postgraduate Medicine 102(3):177–184

Strongylydiasis

� Strongyloidosis

Strübing-Marchiafava-Micheli syndrome

� Paroxysmal nocturnal hemoglob-inuria

Struma-double lips syndrome


Sturge-Weber syndrome

� Nevus flammeus

Stuttgart disease

� Leptospirosis

Subacute cutaneous lupus erythematosus

� Lupus erythematosus, subacute cutaneous

Subacute nodular migratory panniculitis

Synonym(s)Vilanova disease; chronic erythemanodosum; erythema nodosum migrans

DefinitionDisorder characterized by migrating subcu-taneous nodules on the legs, occurringmostly in women

PathogenesisUnknown


Subcorneal pustulosis of Sneddon and Wilkinson 547

S

Clinical manifestationSolitary, discrete, erythematous subcutane-ous nodule or plaque on anterolateral lowerextremity, with peripheral extension later inthe course and without ulceration; addi-tional lesions may occur at other sites overtime

Differential diagnosisErythema nodosum; erythema induratum;lupus panniculitis; traumatic fat necrosis;pancreatic panniculitis; cellulitis

TherapyPotassium iodide: 300 mg PO 3 times daily,increased to 500–1500 mg PO 3–4 times perday as needed; dapsone

ReferencesRoss M, White GM, Barr RJ (1992) Erythematous

plaque on the leg. Vilanova's disease (subacute nodular migratory panniculitis). Archives of Dermatology 128(12):1644–1645, 1647

Subareolar adenomatosis



Synonym(s)Sneddon-Wilkinson disease; subcornealpustulosis of Sneddon and Wilkinson

DefinitionChronic relapsing eruption, characterizedby flaccid pustules that coalesce into largerpustular plaques

PathogenesisNeutrophil chemoattractants, such as inter-leukin 8, leukotriene B4, and complementfragments C5a in lesional skin

Clinical manifestationVariably pruritic, superficial, flaccid pus-tules on normal or minimally erythema-tous skin, typically involving axillae, groin,neck, submammary regions; pus in thelower half of the lesions; lesions isolated orgrouped, and sometimes coalesce to formannular, circinate, or serpiginous plaques;heal with mild hyperpigmentation; furtherwaves of pustulation may arise

Differential diagnosisImpetigo; pustular psoriasis; folliculitis;pemphigus foliaceus; pemphigus vulgaris;dermatitis herpetiformis; bacterial pyo-derma; acute generalized exanthematouspustulosis; dermatophytosis

TherapyDapsone�; corticosteroids, topical, highpotency; acitretin; UVB phototherapy; pho-tochemotherapy

ReferencesReed J, Wilkinson J (2000) Subcorneal pustular

dermatosis. Clinics in Dermatology 18(3):301–313

Subcorneal pustulosis of Sneddon and Wilkinson

� Subcorneal pustular dermatosis

Subcorneal pustular dermatosis. Minimally eroded plaques on the chest wall, abdomen, and arms


548 Subcutaneous fat necrosis

Subcutaneous fat necrosis

� Subcutaneous fat necrosis of new-born

Subcutaneous fat necrosis of newborn

Synonym(s)Panniculitis of the newborn; subcutaneousfat necrosis

DefinitionDisorder characterized by firm, erythema-tous nodules and plaques over the trunk,arms, buttocks, thighs, and cheeks in other-wise healthy newborn infants

PathogenesisPossible causative factors: underlying defectin fat composition or metabolism; neonatalstress resulting in hypothermia with sec-ondary fat crystallization, leading to necro-sis; pressure-induced necrosis occurringduring delivery

Clinical manifestationPresents in normal term neonates as anarea of edema, progressing to variably cir-cumscribed, indurated nodules andplaques; overlying skin sometimes red, pur-ple, or flesh-colored; lesions may becomefluctuant and spontaneously drain necroticfat; antecedant birth trauma (meconiumaspiration, etc.) may precede onset oflesions

Differential diagnosisSclerema neonatorum; cellulitis; erythemanodosum; hemangioma; lipogranulomato-sis (Farber disease); neurofibroma; rhab-domyosarcoma or other sarcoma

TherapySelf-limited process, not requiring therapy

ReferencesBurden AD, Krafchik BR (1999) Subcutaneous fat

necrosis of the newborn: a review of 11 cases. Pediatric Dermatology 16(5):384–387

Subcutaneous fibroma

� Knuckle pads

Subcutaneous phlebitis of the breast and chest wall

� Mondor’s disease

Subungual exostosis

Synonym(s)None

DefinitionAcquired, benign, bony tumor of the distalphalanx, causing overlying nail plate dys-trophy

PathogenesisBegins as a reactive fibrous growth thatdevelops cartilage and ultimately ossifies

Clinical manifestationDistal, subungual mass, usually on the dor-sal-medial great toe; fingernail lesionsrarely occur; overlying nail plate may betented or completely destroyed

Differential diagnosisSquamous cell carcinoma of the nail bed;glomus tumor; subungual wart; melanoma;traumatic nail dystrophy; osteochondroma;enchondroma

TherapySurgical excision


Sunburn 549

S

ReferencesDavis DA, Cohen PR (1996) Subungual exostosis:

case report and review of the literature. Pediat-ric Dermatology 13(3):212–218

Sudamina

� Miliaria


Synonym(s)Exudative discoid and lichenoid dermati-tis; lichenoid chronic dermatosis;polymorphic prurigo syndrome;polymorphous prurigo syndrome; Savill’ssyndrome

DefinitionChronic, pruritic dermatosis, characterizedby discoid, lichenoid, exudative, and urti-carial phases, occurring predominately inadult Jewish men

PathogenesisSuspected to have psychogenic component

Clinical manifestationScrotum and penis are main sites ofinvolvement; discoid phase with round,scaly, and crusted papules; lichenoid phasewith flat-topped, red-violaceosu papulesand plaques; exudative phase with serousexudation from lesions; urticarial phasewith wheals

Differential diagnosisNummular eczema; scabies; atopic dermati-tis; contact dermatitis; lichen planus; lupuserythematosus; dermatitis herpetiformis;mycosis fungoides

TherapyPrednisone�; azathioprine

ReferencesSchmidt H, Midtgaard K (1968) The Sulzberger-

Garbe syndrome. a survey and a case report. Acta Dermato-Venereologica 48(4):287–289

Sun damage


Sun spot

� Lentigo

Sunburn

Synonym(s)Acute sun damage; acute sunburn reaction;erythema solare

DefinitionIntense, transient inflammatory skin reac-tion caused by acute overexposure to ultra-violet radiation in sunlight, primarily ultra-violet B (UV-B)

PathogenesisMost injury from UVB spectrum of sun-light; UV-B absorbed by skin chromo-phores, which become excited and inducemembrane lipid peroxidation and destruc-tion; DNA in epidermal keratinocytesabsorbs ultraviolet light, resulting in pyri-midine dimer formation; synthesis ofcytokines (such as prostaglandins, tumornecrosis factor TNF-α), adhesion mole-cules, histamines, kinins, substance P, calci-tonin gene-related peptide, and nitric oxideinduce tissue injury

Clinical manifestationPersons most prone are those with blue orgreen eye color, lighter skin, and ones who


550 Superficial pemphigus

tan poorly and freckle easily; beginning 2–6hours after excess sun exposure, peaking at15–36 hours, and resolving within 3–5 days;confluent erythema and warmth in exposedareas; edema, pain, and tenderness; pruri-tus with moderate-to-severe sun exposure;vesiculation in severe cases; scaling or peel-ing a few days following exposure; systemicsigns and symptoms such as nausea,abdominal cramping, weakness andmalaise, fever, chills, and headache withsevere sunburn

Differential diagnosisBurn from chemical or heat source; photo-toxic drug eruption; toxic shock syndrome;lupus erythematosus; dermatomyositis;chronic actinic dermatitis; polymorphouslight eruption; erythropoietic protoporphy-ria

TherapyIce water compresses for 20 minutes,repeated 3–4 times daily�; corticosteroids,topical, mid-potency; aloe gel, directly fromplant leaf, applied 3–4 times per day

ReferencesRapaport MJ, Rapaport V (1998) Preventive and

therapeutic approaches to short- and long-term sun damaged skin. Clinics in Dermatolo-gy 16(4):429–439

Superficial pemphigus

� Pemphigus foliaceus

Superficial porokeratosis

� Porokeratosis

Superficial thrombophlebitis

� Thrombophlebitis, superficial

Superficial white onychomycosis

� Onychomycosis

Supernumerary digit

Synonym(s)Rudimentary polydactyly; digital duplica-tion

DefinitionDisorder manifested by a papule on thebase of the ulnar side of the little finger,present from birth

PathogenesisSome cases manifested as autosomal domi-nant trait

Clinical manifestationSmooth, flesh-colored papule at base of thefifth digit, present at birth

Differential diagnosisFibroma; neuroma; neurofibroma; pyo-genic granuloma; wart

TherapySurgical removal for cosmesis only

ReferencesRayan GM, Frey B (2001) Ulnar polydactyly. Plas-

tic & Reconstructive Surgery 107(6):1449–1454


Sweat gland adenoma 551

S

Supernumerary nipple

Synonym(s)Accessory nipple; polythelia

DefinitionCongenital anomaly, characterized by addi-tional nipples and/or related tissue in addi-tion to the two nipples normally appearingon chest

PathogenesisAutosomal dominant transmission withincomplete expressivity; present in somecases of Turner syndrome, Fanconi anemia,ectodermal dysplasia, Kaufman-McKusicksyndrome, and Char syndrome

Clinical manifestationSmall, pigmented or pearl-colored maculeor papule or concave or umbilicated papule,often enlarging at puberty; distributedbilaterally or unilaterally, symmetrically orasymmetrically; usually located along milkline

Differential diagnosisNevocellular nevus; lipoma; lymphangi-oma; neurofibroma; wart; acrochordon

TherapySurgical excision for cosmesis

ReferencesCohen PR, Kurzrock R (1995) Miscellaneous gen-

odermatoses: Beckwith-Wiedemann syn-drome, Birt-Hogg-Dube syndrome, familial atypical multiple mole melanoma syndrome, hereditary tylosis, incontinentia pigmenti, and supernumerary nipples. Dermatologic Clinics 13(1):211–229

Suppurative fasciitis


Suppurative hidradenitis


Suprarenal insufficiency


Sure antiperspirant


Sutton’s nevus

� Halo nevus

Swamp fever

� Leptospirosis

Swamp itch


Sweat gland adenoma



552 Sweating gustatory syndrome

Sweating gustatory syndrome


Sweet syndrome


Sweet’s syndrome


Swimmer’s ear

� Otitis externa

Swimmer’s itch


Swimming pool granuloma


Swineherd's disease

� Leptospirosis

Swollen veins


Sycosis barbae

Synonym(s)Tinea barbae; ringworm of the beard;barber's itch; trichophytosis barbae; tineasycosis

DefinitionSuperficial dermatophyte infection on thebearded areas of the face and neck

PathogenesisHair and hair follicles invaded by fungi,producing inflammatory response; Tricho-phyton species most common and includeT. rubrum, T. mentagrophytes, and T. ver-rucosum

Clinical manifestationInflammatory variant (kerion): causedmostly by zoophilic dermatophytes; soli-tary plaque or nodule, usually localized onchin, cheeks, or neck; inflammatory red-dish papule or nodule with pustules anddraining sinuses, often covered by exudateand crust

Sycosis barbae. Red papules in a beard distribution


Syphilis 553

S

Non-inflammatory variant: erythematousplaques with active border composed ofpapules, vesicles, and/or crusts; hairsbreaking at skin surface or plugging follicle

Differential diagnosisAcne vulgaris; actinomycosis; candidiasis;contact dermatitis; bacterial folliculitis;non-infectious folliculitis; rosacea; halogen-oderma

TherapyTerbinafine; itraconazole; griseofulvin

ReferencesKick G, Korting HC (1998) Tinea barbae due to

Trichophyton mentagrophytes related to per-sistent child infection. Mycoses 41(9–10):439–441

Sycosis cruris

� Tinea cruris

Symmetric progressive leukopathy of extremities


Symmetrical dyschromatosis of the extremities

� Acropigmentation of Dohi

Syndrome of Favre-Racouchot


Synovial cyst


Syphilis

Synonym(s)Lues

DefinitionSexually transmitted or congenital infec-tion caused by the bacterium Treponemapallidum

PathogenesisCaused by the spirochete, Treponema palli-dum; penetrates intact mucous membranesor microscopic dermal abrasions and,within a few hours, enters lymphatics andblood to produce systemic infection; ulti-mate tissue injury related to obliterativeendarteritis

Clinical manifestationPrimary syphilis: occurs within 3 weeks ofcontact with an infected individual; singleulcerated lesion with a surrounding red are-ola; ulcer edge and base have button-likeconsistency; usually heals within 4–8 weeks;painless regional lymphadenopathySecondary syphilis: bilaterally symmetri-cal, pale red to pink, discrete, round mac-

Syphilis. Indurated, red papule on the penis


554 Syringadenoma papilliferum

ules on trunk and proximal extremities;after several days or weeks, appearance ofred, scaly papules sometimes becomingnecrotic; distributed widely, with frequentinvolvement of the palms and soles; smallpapular follicular syphilids involving hairfollicles sometimes result in patchy alo-pecia; highly infectious papules develop atmucocutaneous junctions and in moistintertriginous skin, become hypertrophicand dull pink or gray (condyloma lata);superficial mucosal erosions on the palate,pharynx, larynx, glans penis, vulva, in analcanal and rectum (mucous patches)Late syphilis: usually solitary gummas pre-senting as indurated, nodular, papulosqua-mous or ulcerative lesions forming circlesor arcs with peripheral hyperpigmentation;cardiovascular findings: diastolic murmur,secondary to aortic dilation with valvularinsufficiency; symptomatic neurosyphilis,including meningovascular syphilis: cra-nial nerve palsies and pupillary abnormali-ties occurring with basilar meningitis(Argyll Robertson pupil); tabes dorsalis;ulcers of feet from loss of pain sensationCongenital syphilis: Early manifestations: diffuse eruption,characterized by extensive sloughing of theepithelium, particularly on palms, soles,and skin around mouth and anus; abnor-mal bone radiographs; hepatomegaly;splenomegaly; petechiae; anemia lymphad-enopathy; jaundice; pseudoparalysis; snuf-fles; depressed linear scars radiating fromthe orifice of the mouth (rhagades or Par-rot lines)Late manifestations: interstitial keratitis;cranial nerve VIII deafness; corneal opaci-ties; recurrent arthropathyCongenital neurosyphilis: gummatous peri-ostitis, saddle nose, dental abnormalitiesincluding centrally notched and widelyspaced, peg-shaped, upper central incisors(Hutchinson teeth) and sixth-year molarswith multiple poorly developed cusps (mul-berry molars); bone findings: frontal boss-ing, unilateral irregular enlargement of thesternoclavicular portion of the clavicle

Differential diagnosisAmyloidosis; chancroid; lymphogranulomavenereum; granuloma inguinale; herpessimplex virus infection; drug eruption; ery-thema multiforme; leprosy; tinea corporis;psoriasis; parapsoriasis; lichen planus; pit-yriasis rosea; lupus erythematosus; sar-coidosis; traumatic balanitis

TherapyPenicillin G benzathine�; erythromycin;tetracycline

ReferencesPao D, Goh BT, Bingham JS (2002) Management

issues in syphilis. Drugs 62(10):1447–1461

Syringadenoma papilliferum

� Syringocystadenoma papilliferum

Syringectasia

� Eccrine hidrocystoma

Syringocystadenoma

� Epidermal nevus� Syringocystadenoma papilliferum


Synonym(s)Papillary syringadenoma; nevus syringade-noma papilliferum; syringadenoma papil-liferum; Werther’s tumor, syringocystade-noma


Systemic chondromalacia 555

S

DefinitionBenign tumor, most commonly on thescalp, characterized by one papule, severalpapules in a linear arrangement, or a soli-tary verrucous plaque

PathogenesisTumor with apocrine differentiation; asso-ciated with nevus sebaceous and tubularapocrine adenoma

Clinical manifestationPresents at birth or in early childhood withinfiltrative, verrucous papule or plaque,most commonly on scalp or face; occasion-ally in linear pattern; alopecia over tumorwhen in scalp; at puberty may increase insize and become more papillomatous

Differential diagnosisBasal cell carcinoma; kerion; wart; epider-mal nevus; squamous cell carcinoma


ReferencesMammino JJ, Vidmar DA (1991) Syringocystade-

noma papilliferum. International Journal of Dermatology 30(11):763–766

Syringoma

Synonym(s)None

DefinitionBenign adnexal tumor formed by well-dif-ferentiated sweat ductal elements

PathogenesisMay be related to eccrine elements, apo-crine elements, or pluripotential stem cells

Clinical manifestationSkin-colored or yellowish, small, dermalpapules, often with a translucent or cysticappearance, most commonly on upper

parts of cheeks and lower eyelids, but alsoon axilla, chest, abdomen, penis, and vulva

Differential diagnosisTrichoepithelioma; basal cell carcinoma;molluscum contagiosum; milium; flat wart;xanthelasma; granuloma annulare

TherapySurgical removal for cosmetic reasons only:electrodesiccation and curettage; CO2 laservaporization; dermabrasion; TCA chemicalpeel

ReferencesFrazier CC, Camacho AP, Cockerell CJ (2001) The

treatment of eruptive syringomas in an African American patient with a combination of trichloroacetic acid and CO2 laser destruction. Dermatologic Surgery 27(5):489–492

Systematized elastorrhexis

� Pseudoxanthoma elasticum

Systematized lichenification

� Lichen striatus

Systemic allergic reaction

� Anaphylaxis

Systemic chondromalacia



556 Systemic connective tissue disease

Systemic connective tissue disease


Systemic necrotizing angiitis


Systemic sclerosis


Systemic vasculitis



T


Synonym(s)

Mycosis fungoides

Definition

Heterogenous group of malignant T-celllymphomas with primary manifestations inthe skin

Pathogenesis

Expansion of clone of CD4

+

memory Tcells, which home to the skin


Patch/plaque stage: flat, erythematouspatches, sometimes becoming more infil-trative and evolving into palpable, scalyplaques with irregular borders; alopecia ifscalp is involved

Tumor stage: red-to-violaceous, exophyticand/or ulcerated nodules; generalizederythrodermaPagetoid reticulosis (Woringer-Kolopp dis-ease) variant: solitary, asymptomatic,slowly enlarging, well-defined, red, scalyplaque on the extremitiesSezary variant: large number of circulatingabnormal T cells; erythroderma; lymphade-nopathy


Parapsoriasis en plaque; lymphomatoidpapulosis; psoriasis; lupus erythematosus;lichen planus; atopic dermatitis; tinea cor-poris; other causes of exfoliative erythro-derma, including drug eruption, sebor-rheic dermatitis

Therapy

Topical/physical modalities: corticoster-oids, topical, high potency; UVB photother-apy; photochemotherapy; topical mechlo-rethamine (nitrogen mustard): 90 mg with10 ml of absolute alcohol dissolved in Aqua-phor QS 900 gm, applied daily. Topical car-mustine (BCNU): 100 mg dissolved in 50 mlalcohol; 5 ml dissolved in 50 ml water fordaily application; electron beam therapy;extracorporeal photopheresis.Systemic modalities: prednisone; meth-otrexate; isotretinoin; bexarotene: 20–150 mcg PO per day; systemic chemother-apy

References

Apisarnthanarax N, Talpur R, Duvic M (2002) Treatment of cutaneous T cell lymphoma: cur-

T-cell lymphoma, cutaneous.

Irregular, infiltrated, violaceous plaques on the trunk


558 Taenia solium infestation

rent status and future directions. American Journal of Clinical Dermatology 3(3):193–215

Taenia solium infestation

�

Cysticercosis

Takatsuki syndrome

�

POEMS syndrome

Talon noir

�

Black heel

Tattoo, traumatic

�

Traumatic tattoo

Tay syndrome

Synonym(s)

Trichothiodystrophy with congenital ich-thyosis

;

Tay’s syndrome

;

trichothio-dystro-phy

;

IBIDS

Definition

Hereditary disorder characterized by: pho-tosensitivity; brittle, twisted hair; ichthyo-sis; abnormal fingernails and toenails; mul-tiple developmental defects

Pathogenesis

Autosomal recessive disorder; trichothiod-ystrophy with sulfur-containing amino aciddeficiency in hair; defective repair of UV-

induced DNA lesions possibly associatedwith photosensitivity


Brittle, twisted hair, and ichthyosiformerythroderma; abnormal nails; loss of sub-cutaneous fat, resulting in prematurelyaged-looking face; photosensitivity; slowedphysical development; intellectual impair-ment; wide variety of central nervous sys-tem abnormalities, including seizures,tremors, ataxia and neurosensory deafness;underdeveloped reproductive organs; cata-racts; abnormalities of bones and teeth;increased susceptibility to infection


Progeria; Werner’s syndrome; xerodermapigmentosum; Netherton’s syndrome; Sjö-gren-Larsson syndrome; Cockayne’s syn-drome; non-bullous ichthyosiform erythro-derma

Therapy

Emollients for dry skin

References

Kousseff BG, Esterly NB (1988) Trichothiodystro-phy, IBIDS syndrome or Tay syndrome? Birth Defects: Original Article Series 24(2):169–181, 1988

Tay’s syndrome

�

Tay syndrome

Tazarotene

Trade name(s)

Tazorac

Generic available

No

Drug class

Retinoid


Telogen effluvium 559

T

Mechanism of action

Gene transcription after membrane recep-tor binding and intracellular transport;modulates abnormal epidermal keratiniza-tion

Dosage form

0.05%, 0.1% gel and cream


See table

Common side effects

Cutaneous:

scaling, erythema, blistering,photosensitivity


None

Drug interactions

Benzoyl peroxide; isotretinoin; photosensi-tizing drugs



References

Tremblay JF, Bissonnette R (2002) Topical agents for the treatment of psoriasis, past, present and future. Journal of Cutaneous Medicine & Sur-gery 6(3 Suppl):8–11

Telangiectasia macularis eruptiva perstans

�

Mastocytosis

Telogen defluvium

�

Telogen effluvium

Telogen effluvium

Synonym(s)

Telogen defluvium

Definition

Reactive process resulting in nonscarringalopecia, characterized by diffuse hair shed-ding, caused by metabolic or hormonalstress or by medications

Pathogenesis

Large number of hairs entering telogenphase at one time; shedding occurs whennew anagen hairs begin to grow; emerginghairs force some of the resting hairs out ofthe follicle, leading to temporary alopecia


Acute form: relatively sudden onset of dif-fuse scalp hair loss, usually after a meta-bolic or physiologic stress 1–6 monthsbefore the start of the hair shedding; incit-ing stresses: febrile illness, major injury,change in diet, pregnancy and delivery, andbeginning a new medicationChronic form: hair shedding lasting longerthan 6 months; onset often insidious; incit-ing causes: chronic illness such as malig-nancy, particularly lymphoproliferativemalignancy; any chronic debilitating ill-ness such as systemic lupus erythematosus;

Tazarotene. Dermatologic indications and dosage


Acne vulgaris Apply daily Apply daily

Psoriasis Apply daily Apply daily

Reiter syndrome Apply daily Apply daily

Sun-induced skin aging

Apply daily Apply daily


560 Temporal arteritis

end-stage renal disease or liver disease; hor-monal changes; diet changes; heavy metalintoxication


Alopecia areata; androgenetic alopecia; tri-chotillomania; tinea capitis; anagen efflu-vium; traumatic hair breakage

Therapy

Minoxidil 5% solution 1 ml applied twicedaily

References

Sperling LC (2001) Hair and systemic disease. Dermatologic Clinics 19(4):711–726

Temporal arteritis

Synonym(s)

Giant cell arteritis

;

arteritis temporalis

;

arteritis cranialis

;

Horton disease

;

granulomatous arteritis

;

arteritis of theaged

Definition

Vasculitis that affects large and medium-sized arteries containing elastic tissuethroughout the body, most commonly thetemporal arteries

Pathogenesis

Vasculitis primarily damaging the mediaand destroying the internal elastic layer;panarteritis developing and intimal prolif-eration causing lumenal occlusion, result-ing in signs and symptoms of decreasedperfusion


Constitutional symptoms, such as malaise,weight loss, fever and fatigue; temporalheadache; tender scalp; jaw claudication;visual changes, including diplopia, blurredvision, amaurosis fugax, and blindness ofone or both eyes; temporal arteries may be

tender, inflamed, dilated, thickened orcord-like, and pulsatile; ulceration some-times occurring over the temporal artery


Wegener’s granulomatosis; amyloidosis;polymyalgia rheumatica; polyarteritisnodosa; lupus erythematosus; rheumatoidarthritis; Takayasu arteritis

Therapy

Prednisone

�

References

Salvarani C, Cantini F, Boiardi L, Hunder GG (2002) Polymyalgia rheumatica and giant-cell arteritis. New England Journal of Medicine 347(4):261–271

Tendinous xanthoma

�

Xanthoma

Tennis heel

�

Black heel

Tennis toe

Synonym(s)

Sportsman’s toe

Definition

Acute subungual accumulation of bloodfrom sudden blunt impact of the toe againstathletic footwear

Pathogenesis

Subungual hemorrhage of the lateral nailbed caused by jamming of the toe into thefront of the athletic shoe


Tennis toe 561

T


Pain and reddish-blue discoloration underthe affected nail plate; mainly occurs insports that require frequent, abrupt stops

and quick pivoting, such as basketball, ten-nis, squash, and racquetball; usually affectseither the great toe or second toe, which-ever is longer; sometimes occurs with jog-

Terbinafine. Dermatologic indications and dosage


Black piedra 250 mg PO once daily for 1 month 250 mg PO for 1 month (> 40 kg weight); 125 mg PO for 1 month (20–40 kg weight); 62.5 mg PO daily for 1 month (< 20 kg weight)

Chromoblastomycosis 250 mg PO daily for 3-6 months 250 mg PO 3-6 months (> 40 kg weight); 125 mg PO for 3-6 months (20–40 kg weight); 62.5 mg PO daily for 3-6 months (< 20 kg weight

Majocchi granuloma 250 mg PO daily for 4–6 weeks 250 mg PO for 4–6 weeks (> 40 kg weight); 125 mg PO for 4–6 weeks (20–40 kg weight); 62.5 mg PO daily for 4–6 weeks (< 20 kg weight)

Onychomycosis 250 mg PO daily for 3 months 250 mg PO for 3 months (> 40 kg weight); 125 mg PO for 3 months (20–40 kg weight); 62.5 mg PO daily for 3 months (< 20 kg weight)

Sycosis barbae 250 mg PO daily for 4 weeks 250 mg PO for 4 weeks (> 40 kg weight); 125 mg PO for 4 weeks (20–40 kg weight); 62.5 mg PO daily for 4 weeks (< 20 kg weight

Tinea capitis 250 mg PO daily for 4–6 weeks 250 mg PO for 4–6 weeks (> 40 kg weight); 125 mg PO for 4–6 weeks (20–40 kg weight); 62.5 mg PO daily for 4-6weeks (< 20 kg weight)

Tinea corporis 250 mg PO daily for 4–6 weeks 250 mg PO for 4–6 weeks (> 40 kg weight); 125 mg PO for 4-6 weeks (20–40 kg weight); 62.5 mg PO daily for 4–6 weeks (< 20 kg weight)

Tinea cruris Apply cream twice daily for 7 days or 250 mg PO for 2–4 weeks

Apply cream twice daily for 7 days

Tinea faciei Apply cream twice daily for 7 days or 250 mg PO for 2–4 weeks

Apply cream twice daily for 7 days or250 mg PO for 2–4 weeks (> 40 kg weight); 125 mg PO for 2–4 weeks (20–40 kg weight); 62.5 mg PO daily for 2–4 weeks (< 20 kg weight)

Tinea nigra Apply cream twice daily for 7 days or 250 mg PO for 2–4 weeks


Tinea pedis Apply cream twice daily for 7 days or 250 mg PO for 2–4 weeks



562 Terbinafine

ging, affecting the third, fourth, or fifthtoes, secondary to repeated pounding of thefoot on a firm running surface


Melanoma; melanocytic nevus

Therapy

Puncture of the nail plate with a bluntpointed instrument, such as a heated paperclip, to express blood

�

References

Elizabeth L. Tanzi, MD, Richard K. Scher (1999) Managing common nail disorders in active pa-tients and athletes. Physician and Sportsmedi-cine 27(9):35–37

Terbinafine

Trade name(s)

Lamisil

Generic available

No

Drug class

Allylamine antifungal agent

Mechanism of action

Inhibition of squalene epoxidase, whichblocks ergosterol synthesis

Dosage form

250 mg tablet; 1% cream


See table

Common side effects

Cutaneous:

skin eruption, pruritus (oralformulation)

Gastrointestinal:

nausea and vomiting,diarrhea, dyspepsia

Laboratory:

elevated liver enzymes

Neurologic:

taste changes


Cutaneous:

Stevens-Johnson syndrome,toxic epidermal necrolysis, anaphylaxis

Gastrointestinal:

hepatotoxicity

Laboratory:

elevated liver enzymes, neutro-penia

Drug interactions

Cimetidine; cyclosporine; rifampin; theo-phylline; thioridazine; tricyclic antidepres-sants


Hypersensitivity to drug class or compo-nent; caution in patients with impaired liveror renal function

References

Moosavi M, Bagheri B, Scher R (2001) Systemic antifungal therapy. Dermatologic Clinics 19(1):35–52

Terminal transverse defects of arm

�

Amniotic band syndrome

Tetracycline

Trade name(s)

Sumycin; Achromycin-V; Tetracap; Panmy-cin

Generic available

Yes

Drug class

Tetracycline antibiotic


Tetracycline 563

T

Tetracycline. Dermatologic indications and dosage


Acne vulgaris 250–500 mg PO twice daily Not indicated below age 8 years; 250–500 mg PO twice daily


250–500 mg PO twice daily Not indicated below age 8 years; 250–500 mg PO twice daily

Bejel 500 mg PO 4 times daily for 15 days Not indicated below age 8 years; 250 mg PO 4 times daily for 15 days

Bullous pemphigoid 250–500 mg PO twice daily Not indicated below age 8 years; 250–500 mg PO twice daily

Chloracne 250–500 mg PO twice daily Not indicated below age 8 years; 250–500 mg PO twice daily



Folliculitis 250–500 mg PO twice daily Not indicated below age 8 years; 250–500 mg PO twice daily

Glanders 40 mg per kg daily, divided into 3 doses for 60–150 days

Not indicated below age 8 years; 40 mg per kg daily, divided into 3 doses for 60–150 days





Melioidosis 40 mg per kg daily, divided into 3 doses for 60–150 days

Not indicated below age 8 years; 40 mg per kg daily, divided into 3 doses for 60–150 days

Perioral dermatitis 250–500 mg PO twice daily Not indicated below age 8 years; 250–500 mg PO twice daily

Pinta 500 mg PO 4 times daily for 15 days Not indicated below age 8 years; 25-50 mg per kg daily PO for 15 days

Pityriasis lichenoides 250–500 mg PO twice daily Not indicated below age 8 years; 250–500 mg PO twice daily

Protothecosis 500 mg PO twice daily for 1-6 weeks (combined with amphotericin B)

Not indicated below age 8 years; 250 mg PO twice daily for 1-6 weeks (combined with amphotericin B)

Relapsing fever 1 gm PO twice daily for 7 days after patient becomes afebrile

Not indicated below age 8 years; 500 mg PO twice daily for 7 days after patient becomes afebrile

Rhinoscleroma 500 mg PO twice daily for months to years

Not indicated below age 8 years; 500 mg PO twice daily for months to years

Rickettsialpox 500 mg PO twice daily for 5 days Not indicated below age 8 years; 250 mg PO twice daily for 5 days

Rosacea 250–500 mg PO daily Not indicated below age 8 years; 250–500 mg PO twice daily


564 Thalidomide

Mechanism of action

Antibiotic activity: protein synthesis inhibi-tion by binding to the 30S ribosomal subu-nitAnti-inflammatory activity: unclear mecha-nism

Dosage form

250 mg, 500 mg capsule


See table

Common side effects

Cutaneous:

photosensitivity, stomatitis,oral candidiasis, urticaria or other vascularreaction

Gastrointestinal:

nausea and vomiting,diarrhea, esophagitis

Neurologic:

tinnitus, dizziness, drowsiness,headache, ataxia


Gastrointestinal:

pseudomembranous coli-tis, hepatotoxicity

Neurologic:

pseudotumor cerebri

Hematologic:

neutropenia, thrombocytope-nia


Contraindications/precautionsHypersensitivity to drug class or compo-nent; pregnancy; patient < 8 years old; cau-tion in patients with impaired renal or liverfunction



Thalidomide

Trade name(s)Thalomid

Generic availableNo


Mechanism of actionImmunomodulatory; anti-inflammatory;hypnotic-sedative



Steatocystoma multiplex


Syphilis 500 mg PO 4 times daily for 14 days; late latent syphilis with normal CSF examination, cardiovascular syphilis, and late benign (gumma) disease – 500 mg PO 4 times daily for 28 days

Not indicated


1 gm twice daily until ulcer closure Not indicated below age 8 years; 500 mg PO twice daily until ulcer closure

Tetracycline. Dermatologic indications and dosage (Continued)



Thioguanine 565

T

Common side effectsCutaneous: eruption, photosensitivityConstitutional: fever, chillsGastrointestinal: increased appetite andweight gain, diarrheaNeurologic: somnolence, mood changes,confusion, amnesia, headache

Serious side effectsBone marrow: neutropeniaCardiovascular: severe hypertension,bradycardiaCutaneous: Stevens-Johnson syndrome,toxic epidermal necrolysisNeurologic: peripheral neuropathy, seizuresPregnancy: severe birth defects

Drug interactionsAcetaminophen; antihistamines; antipsy-chotics; barbiturates; protease inhibitors;griseofulvin; rifampin; phenytoin; car-bamazepine; opiates; sedative hypnotics


Contraindications/precautionsHypersensitivity to drug class or compo-nent; pregnancy; moderate to severe pre-

existing peripheral neuropathy; caution inpatients with seizure disorder, cardiovascu-lar disease, or child-bearing potential

ReferencesRadomsky CL, Levine N (2001) Thalidomide.

Dermatologic Clinics 19(1):87–103

Therapy-induced bullous photosensitivity

� Pseudoporphyria

Thioguanine

Trade name(s)Thioguanine

Generic availableNo

Drug classPurine analog

Thalidomide. Dermatologic indications and dosage



100 mg PO daily for 2 months Not indicated

Leprosy, reactional state










Prurigo nodularis 100–300 mg PO daily Not indicated


566 Thost-Unna disease

Mechanism of actionInhibition of lymphocyte synthesis



Common side effectsGastrointestinal: diarrhea, nausea, vomit-ingNeurologic: headache, fatigue

Serious side effectsBone marrow: myelosuppressionGastrointestinal: hepatotoxicity

Drug interactionsSulfasalazine; busulfan; azathioprine

Contraindications/precautionsHypersensitivity to drug class or compo-nent; caution with other immunosuppres-sants or bone marrow suppressants

ReferencesSilvis NG, Levine N (1999) Pulse dosing of thio-

guanine in recalcitrant psoriasis. Archives of Dermatology 135(4):433–437

Thost-Unna disease


Three day measles

� Rubella

Thrombophlebitis, superficial

Synonym(s)Venous clot

DefinitionInflammatory reaction with thrombus of asubcutaneous vein

PathogenesisAssociated with intimal damage (fromtrauma, infection, or inflammation), stasis,or changes in blood constituents; risk fac-tors: varicose veins, obesity, old age, ciga-rette smoking, and injection of causticmaterials such as street drugs

Clinical manifestationRedness and tenderness along course ofvein, usually accompanied by edema; oftenoccurs in patients with varicose veins;involvement of upper extremities at infu-sion sites or sites of trauma

Differential diagnosisDeep vein thrombophlebitis; cellulitis; facti-tial disease; lymphangitis; pancreatic pan-niculitis; Weber-Christian disease; lupus

Thioguanine. Dermatologic indications and dosage


Pityriasis rubra pilaris 120 mg PO twice weekly to 160 mg PO 3 times weekly

Not indicated

Psoriasis 120 mg PO twice weekly to 160 mg PO 3 times weekly

Not indicated

Reiter syndrome 120 mg PO twice weekly to 160 mg PO 3 times weekly

Not indicated


Tinea barbae 567

T

panniculitis; erythema nodosum; ery-thema induratum

TherapyElastic support dressings; for severeinvolvement: bedrest with elevation of theextremity and application of hot, wet com-presses

ReferencesKalodiki E, Nicolaides AN (2002) Superficial

thrombophlebitis and low-molecular-weight heparins. Angiology 53(6):659–663

Thrush

� Candidiasis

Thyroglossal duct cyst

� Cutaneous columnar cyst

Thyroid acropachy

DefinitionClubbing of fingers and toes, associatedwith soft tissue thickening and periostealnew bone formation of distal hands and feetin patients with hyperthyroidism

ReferencesNiepomniszcze H, Amad RH (2001) Skin disor-

ders and thyroid diseases. Journal of Endo-crinological Investigation 24(8):628–638

Thyroid blepharochalasis syndrome


Tick bite fever


Tick fever


Tick typhus

� Boutonneuse fever� Rocky Mountain spotted fever

Tick-borne relapsing fever

� Relapsing fever

Tinea amiantacea

DefinitionMorphologic entity characterized by thick,adherent scale on the scalp and in the hair

ReferencesBettencourt MS, Olsen EA (1999) Pityriasis ami-

antacea: a report of two cases in adults. Cutis 64(3):187–189

Tinea barbae

� Sycosis barbae


568 Tinea capitis

Tinea capitis

Synonym(s)Ringworm of the scalp

DefinitionSuperficial fungal infection of scalp skin,eyebrows, and eyelashes, with propensityfor invading hair shafts and follicles

PathogenesisCausative agents are fungal species of gen-era Trichophyton and Microsporum; afterinoculation, fungal hyphae invades hairkeratin as it is formed

Clinical manifestationRed papules progressing to grayish, annu-lar plaques consisting of perifollicularpapules; pustules with crusts, exudate, mat-ted hairs, and debrisBlack dot variant: infection with fracture ofthe hair, leaving dark stubs visible in thefollicular orificesKerion variant: extreme inflammation asso-ciated with boggy scalp and pustules; mayprogress to patchy or diffuse hair loss withscarring alopeciaFavus variant (tinea favosa): chronic infec-tion caused most commonly by T. schoen-leinii, characterized by yellow, cup-shapedcrusts, termed scutula, which surround theinfected hair follicles

Differential diagnosisPsoriasis; seborrheic dermatitis; pediculo-sis; alopecia areata; traction alopecia; tri-chotillomania; folliculitis; secondary syphi-lis; bacterial pyoderma

TherapyGriseofulvin; itraconazole; terbinafine;prednisone for kerion�

ReferencesAl Sogair S, Hay RJ (2000) Fungal infection in

children: tinea capitis. Clinics in Dermatology 18(6):679–685

Tinea corporis

Synonym(s)Ringworm

DefinitionSuperficial fungal infection of glabrous skinof the trunk and extremities

PathogenesisCausative agent mainly fungal species ofgenera Microsporum (most commonly M.canis) and Trichophyton (most commonlyT. rubrum); pathogens produce kerati-nases, allowing invasion of stratum cor-neum; cell wall of T rubrum contains man-nan, possible inhibitor of local cell-medi-ated immunity; infection transmitted byfomites, such as infected pets, or by autoin-oculation from reservoir elsewhere on skin

Clinical manifestationRapidly evolving, annular, erythematous,scaly plaques; border may have crusting,vesicle formation, and papules; intenseinflammatory response with zoophilic fungi(e.g., M. canis)Majocchi granuloma variant: granuloma-tous reaction secondary to fungal folliculi-tis, usually caused by T rubrum; plaquesstudded with follicular papules and/or pus-

Tinea capitis. Boggy, red, alopecic plaque, studded with papules and minute pustules


Tinea faciei 569

T

tules; organism also occurs in the sur-rounding dermisTinea manuum variant: diffuse erythemaand scale of palm, extending onto dorsumof handTinea imbricata variant: caused by T con-centricum; scaly plaques arranged in con-centric rings

Differential diagnosisTinea versicolor; psoriasis; seborrheic der-matitis; lupus erythematosus; bacterial pyo-derma; candidiasis; contact dermatitis;superficial pemphigus; pityriasis rosea;syphilis; nummular eczema; granulomaannulare; sarcoidosis; erythema annularecentrifugum

TherapyAzole antifungal agents; ciclopirox; terbin-afine cream; griseofulvin; itraconazole; oralterbinafine

ReferencesLesher JL Jr (1999) Oral therapy of common su-

perficial fungal infections of the skin. Journal of the American Academy of Dermatology 40(6 Pt 2):S 31–34

Tinea cruris

Synonym(s)Tinea inguinalis; groin dermatophytosis;ringworm of the groin; eczema mar-ginatum; gym itch; jock itch; crotch rot

DefinitionSuperficial fungal infection of groin andadjacent skin

PathogenesisCausative agents the fungal species of gen-era Trichophyton (most commonly, T.Rubrum) and Epidermophyton; pathogensproduce keratinases, allowing invasion ofstratum corneum; infection transmitted byfomites, such as contaminated towels, or by

autoinoculation from reservoir elsewhereon skin

Clinical manifestationAlmost exclusively in men; erythema withcentral clearing with hyperpigmentationand advancing scaly border in inguinalcreases; extends distally onto medial thighsand proximally to lower abdomen andpubic area; with acute infections, moistureand exudation; scrotum spared

Differential diagnosisPsoriasis; seborrheic dermatitis; pediculo-sis; bacterial pyoderma; candida intertrigo;contact dermatitis; acanthosis nigricans;erythrasma; benign familial pemphigus;Langerhans cell histiocytosis


ReferencesWeinstein A, Berman B (2002) Topical treatment

of common superficial tinea infections. Ameri-can Family Physician 65(10):2095–2102

Tinea faciei

Synonym(s)Ringworm of the face; facial ringworm

DefinitionSuperficial fungal infection of glabrous skinof face

PathogenesisCausative agent mainly the fungal species ofgenera Microsporum (most commonly, M.canis) and Trichophyton (most commonly,T. tonsurans); pathogens produce kerati-nases, allowing invasion of stratum cor-neum; infection transmitted by fomites,such as infected pets, or by autoinoculationfrom reservoir elsewhere on skin


570 Tinea favosa

Clinical manifestationPruritic, annular, or serpiginous erythema-tous scaling plaques, with active bordercomposed of papules, vesicles, and/orcrusts

Differential diagnosisPsoriasis; seborrheic dermatitis; lupus ery-thematosus; bacterial pyoderma; candidia-sis; contact dermatitis; superficial pemphi-gus; rosacea; perioral dermatitis; coccidio-idomycosis; granuloma annulare;sarcoidosis



perficial fungal infections of the skin. Journal of the American Academy of Dermatology 40(6 Pt 2):S 31–34

Tinea favosa

� Tinea capitis

Tinea flava


Tinea imbricata

� Tinea corporis

Tinea inguinalis

� Tinea cruris

Tinea manuum

� Tinea corporis

Tinea nigra

Synonym(s)Tinea nigra palmaris; tinea nigra plantaris;keratomycosis nigricans palmaris;dermatomycosis nigricans

DefinitionSuperficial fungal infection, characterizedby hyperpigmented macules or patches,usually occurring on palms

PathogenesisCausative agent the fungal pathogen, Phae-oannellomyces werneckii; inoculation fromcontamination source such as soil, sewage,wood, or compost; pigmentary change dueto accumulation of melanin-like substancein fungus

Clinical manifestationDiscrete, oval, round or irregular, painless,brown-to-black macule or patch, beginningas small dark spot; hyperpigmentationranging from light brown to black andsometimes appearing mottled or velvety;varies in size, depending on the duration ofinfection

Differential diagnosisExogenous staining; melanoma; yaws;pinta; drug-induced hyperpigmentation

TherapyAzole antifungal agents; ciclopirox; terbin-afine cream

ReferencesShannon PL, Ramos-Caro FA, Cosgrove BF, Flow-

ers FP (1999) Treatment of tinea nigra with ter-binafine. Cutis 64(3):199–201


Tinea unguium 571

T

Tinea nigra palmaris

� Tinea nigra

Tinea nigra plantaris

� Tinea nigra

Tinea nodosa

� Piedra

Tinea pedis

Synonym(s)Ringworm of the feet; athlete’s feet

DefinitionSuperficial fungal infection of the skin ofthe feet

PathogenesisCausative agent the fungal species of gen-era Epidermophyton (most commonly, E.floccosum) and Trichophyton (most com-monly, T. rubrum or T. mentagrophytes);pathogens produce keratinases, allowinginvasion of stratum corneum; cell wall of Trubrum contains mannan, possible inhibi-tor of local cell-mediated immunity; tem-perature and serum factors, such as betaglobulins and ferritin, may play role in lim-iting infection; hyperhidrosis a risk factorfor infection

Clinical manifestationInterdigital variant: maceration, fissuring,and scaling, most often between fourth and

fifth toes; usually spares dorsal aspect offoot, but some extension onto plantar sur-faceMoccasin (hyperkeratotic) variant: sym-metrical, asymptomatic or pruritic ery-thema with slight scaling; dorsal footspared, but sometimes extends onto thesides of the footVesicular variant: painful, pruritic vesiclesor bullae, most often on instep or anteriorplantar surfaces; clear or purulent fluid inblisters; after rupture, scaling with ery-themaUlcerative variant: rapidly spreading vesic-ulopustular lesions, often with secondarybacterial infection; may develop cellulitis,lymphangitis, pyrexia, and malaise

Differential diagnosisPsoriasis; dyshidrotic eczema; atopic der-matitis; bacterial pyoderma; candidiasis;contact dermatitis; erythema multiforme;syphilis; localized bullous pemphigoid;xerosis



perficial fungal infections of the skin. Journal of the American Academy of Dermatology 40 (6 Pt 2):S 31–34

Tinea sycosis

� Sycosis barbae

Tinea unguium

� Onychogryphosis


572 Tinea versicolor

Tinea versicolor

Synonym(s)Pityriasis versicolor; chromophytosis;dermatomycosis furfuracea; tinea flava

DefinitionSuperficial fungal infection, characterizedby hypopigmented or hyperpigmentedmacules, patches, and scaly papules on thechest, neck, and back

PathogenesisCaused by dimorphic, lipophilic organism,Malassezia furfur, normal constituent ofhost flora in yeast form; factors associatedwith conversion to mycelial morphologicform: genetic predisposition; warm, humidenvironments; immunosuppression; malnu-trition; Cushing disease; individual varia-tions in skin surface lipids may be factor indisease susceptibility

Clinical manifestationWell-marginated, reticulated, finely scaly,oval-to-round, variably colored papules,coalescing into plaques; located over trunk,neck, chest, with occasional extension toabdomen and proximal extremities; morenoticeable during summer months; inimmunosuppressed patients, lesions in flex-ural regions, face, or isolated areas ofextremities

Differential diagnosisTinea corporis; parapsoriasis; psoriasis;confluent and reticulated papillomatosis ofGougerot and Carteaud; erythrasma; pityr-iasis alba; seborrheic dermatitis; vitiligo

TherapyKetoconazole; azole antifungal agents;ciclopirox; terbinafine cream; seleniumsulfide 2.5 % lotion applied every other dayfor 2 weeks

ReferencesGupta AK, Bluhm R, Summerbell R (2002) Pityr-

iasis versicolor. Journal of the European Acad-emy of Dermatology & Venereology 16(1):19–33

Toasted skin syndrome


Tomato tumor

� Cylindroma

Toriello-Carey syndrome

Synonym(s)Corpus callosum agenesis-facial anomalies-Robin sequence syndrome

DefinitionCongenital syndrome consisting of agene-sis of the corpus callosum, multiple facialdefects, laryngeal abnormalities, heartdefect, skeletal anomalies, and developmen-tal delay

PathogenesisMay have X-linked inheritance

Clinical manifestationAgenesis of the corpus callosum; telecan-thus; short palpebral fissures; small nosewith anteverted nares; malformed ears;redundant neck skin; laryngeal abnormali-ties; heart defect; short hands; hypotonia;occasional Hirschsprung disease, moderateto severe developmental delay

Differential diagnosisNone


Toxic epidermal necrolysis 573

T


ReferencesCzarnecki P, Lacombe D, Weiss L (1996) Toriello-

Carey syndrome: evidence for X-linked inher-itance. American Journal of Medical Genetics 65(4):291–294

Torre syndrome

� Muir-Torre syndrome

Torulosis

� Cryptococcosis

Touraine-Solente-Gole syndrome

� Pachydermoperiostosis

Toxemic erythema of pregnancy


Toxemic rash of pregnancy


Toxic epidermal necrolysis

Synonym(s)Acute disseminated epidermal necrosis;acute skin failure; Lyell syndrome

DefinitionSevere, acute, systemic disorder character-ized by extensive epidermal loss

PathogenesisMost often drug-induced (antiepilepticdrugs, sulfonamides, ampicillin, allopuri-nol, nonsteroidal anti-inflammatoryagents); immune-related cytotoxic reactiondestroys keratinocytes; TNF-α likely mainmediator in epidermal destruction directlythrough apoptosis, indirectly by stimulat-ing cytotoxic T cells

Clinical manifestationProdrome of malaise, fever, cough, sorethroat, myalgia, rhinitis, and anorexia; skinlesions beginn as morbilliform eruption;epidermal sloughing in sheets, leavingmoist, denuded dermis; positive Nikolskysign; hemorrhagic crusting of the lips; con-junctivitis; pneumonia is a major complica-tion

Differential diagnosisToxic shock syndrome; Stevens-Johnsonsyndrome; Kawasaki disease; staphylococ-

Toxic epidermal necrolysis. Full-thickness epidermal sloughing


574 Toxic erythema

cal scalded skin syndrome; exfoliativeerythroderma; bullous pemphigoid; pem-phigus vulgaris; chemical or thermal burn

TherapyDiscontinuation of all suspect medica-tions�; intravenous immunoglobulin(IVIG) – 2 gm per kg IV given over 3 days;plasmapheresis


(2002) Emergency department dermatology. Current Problems in Dermatology 14(6):188–220

Toxic erythema


Toxic erythema of newborn


Toxic oil syndrome

Synonym(s)Spanish toxic oil syndrome

DefinitionIllness resulting from consumption of adul-terated rapeseed oil, characterized byintense myalgias, marked peripheral eosi-nophilia, pulmonary infiltrates, and sclero-derma-like skin changes

PathogenesisSuggestion of autoimmune mechanisms;directly related to consumption of oils con-taining fatty acid esters of 3-(N-phe-nylamino)-1,2-propanediol (PAP)

Clinical manifestationProdrome of fever, headache, cough, dysp-nea, and pruritus; after 1 month, develop-ment of extremity edema, followed by scle-roderma-like changes; extreme myalgiaswith subsequent muscle atrophy; late alo-pecia, sicca syndrome, and liver abnormali-ties; peripheral eosinophilia; chronicchanges more common in women

Differential diagnosisEosinophilia-myalgia syndrome; progres-sive systemic sclerosis; eosinophilic fascii-tis; dermatomyositis; hypereosinophilicsyndrome


ReferencesDiggle GE (2001) The toxic oil syndrome: 20 years

on. International Journal of Clinical Practice 55(6):371–375

Toxic shock syndrome

Synonym(s)Staphylococcal toxic shock syndrome

DefinitionAcute febrile illness, characterized by gen-eralized erythematous eruption accompa-nied by hypotension and multiple organdysfunction

PathogenesisCaused by strains of Staphylococcus aureusproducing TSS toxin, associated withrelease of tumor necrosis factor- α (TNF-α)and interleukin-1 (IL-1), which mediatesigns and symptoms of disease; predispos-ing factors: influenza, sinusiitis, intrave-nous drug use, HIV infection, burn or otherwounds, postoperative infection

Clinical manifestationSkin and mucous membrane changes: dif-fuse macular erythroderma or scarletini-


Traction alopecia 575

T

form eruption; erythema and edema ofpalms and soles; hyperemia of conjunctivaand mucous membranes, with strawberrytongue; delayed palm and sole desquama-tionOther organ involvement: fever; hypoten-sion; cardiomyopathy; nausea; vomiting;diarrhea; rhabdomyolysis; myalgias; mus-cle tenderness and weakness; azotemia;acute renal failure; adult respiratory dis-tress syndrome; elevated serum glutamicoxaloacetic transaminase (SGOT) andserum bilirubin; thrombocytopenia; leuko-cytosis; disseminated intravascular coagu-lation; hypophosphatemia; hypocalcemia;electrolyte imbalance

Differential diagnosisStreptococcal toxic shock-like syndrome;Kawasaki disease; staphylococcal scaldedskin syndrome; toxic epidermal necrolysis;drug reaction; scarlet fever; Rocky Moun-tain spotted fever; leptospirosis

TherapyNafcillin: 1–2 gm IV every 4 hours in adults;50–200 mg per kg per day divided into 4–6doses per day in childrenClindamycin: 600–900 mg IV every 8 hoursin adults; 20–40 mg per kg per day IVdivided into 3–4 doses in children


(2002) Emergency department dermatology. Current Problems in Dermatology 14(6):188-220

Trabecular carcinoma


Trachyonychia

� Twenty nail dystrophy

Traction alopecia

Synonym(s)Traumatic alopecia marginalis; pressurealopecia; massage alopecia; ponytail bandalopecia

DefinitionGroup of acute or chronic scalp injuriesleading to patchy alopecia

PathogenesisExcessive traction for prolonged periods(e.g., tight braiding, wearing of ponytails)causes conversion of anagen phase to telo-gen phase hair growth; overprocessing,chemical treatment of hair with dyes,bleaches, or straighteners disrupts keratinstructure and reduces its tensile strength,making it susceptible to breakage

Clinical manifestationPatchy areas of hair loss; hair-pulling testresults in the detachment of more than 6strands; may have perifollicular erythema,scaling, and pustules; marginal alopecia intemporal region or occipital area; withcornrowing hair style, most affected areaimmediately adjacent to the braided region;reversible if causitive hair styling practicediscontinued early in course

Differential diagnosisAlopecia areata; androgenetic alopecia; tri-chotillomania; tinea capitis; folliculardegeneration syndrome; telogen effluvium;anagen effluvium; syphilis; lupus erythema-tosus

TherapyDiscontinuation of practices that exert trac-tion on hair or otherwise traumatize hair�

ReferencesSperling LC, Mezebish DS (1998) Hair diseases.



576 Transient acantholytic dermatosis


Synonym(s)Grover disease; Grover’s disease

DefinitionPruritic papular disorder, characterizedhistologically by focal acantholysis

PathogenesisAssociation with heat and sweating

Clinical manifestationPruritic eruption of the skin of the anteriorchest, upper back, and lower rib cage; mul-tiple, discrete, erythematous to red-brownkeratotic papules, most common in middle-aged or older men; occasionally, lesions areacneiform, vesicular, or pustular

Differential diagnosisFolliculitis; Darier’s disease; Hailey-Haileydisease; pemphigus foliaceus; insect bitereaction; scabies; dermatitis herpetiformis;tinea corporis; psoriasis; xerotic eczema;pityriasis rosea; miliaria; drug eruption

TherapyVitamin A 150,000 units PO daily for 30days, repeated after a 1 month rest period;isotretinoin

ReferencesParsons JM (1996) Transient acantholytic derma-

tosis (Grover's disease): a global perspective. Journal of the American Academy of Dermatol-ogy 35(5 Pt 1):653–666

Transient bullous dermolysis of newborn


Transient neonatal pustular melanosis

Synonym(s)None

DefinitionDisorder usually present at birth, character-ized by vesicles, superficial pustules, andpigmented macules

PathogenesisUnknown

Clinical manifestationPustules and pigmented macules foundmainly on the chin, neck, or forehead,behind the ears, or on the trunk, palms, andsoles; no systemic signs or symptoms; mostcommon in black neonates

Differential diagnosisMongolian spot; acropustulosis of infancy;erythema toxicum neonatorum; neonatalherpes simplex virus infection; miliaria;milia; neonatal acne; impetigo; candidiasis


ReferencesVan Praag MC, Van Rooij RW, Folkers E, Spritzer

R, Menke HE, Oranje AP (1997) Diagnosis and

Transient acantholytic dermatosis. Multiple red, scaly, or eroded papules on the trunk


Trench fever 577

T

treatment of pustular disorders in the neonate. Pediatric Dermatology 14(2):131–143

Transient neonatal pustulosis

� Transient neonatal pustular mela-nosis

Transient symptomatic zinc deficiency


Traumatic alopecia marginalis


Traumatic tattoo

Synonym(s)Accidental tattoo

DefinitionLocalized skin dyspigmentation secondaryto deposition of colored material in the skinfrom a deep dirty abrasion or other pene-trating injury

PathogenesisDeposition of material into dermis, oftenafter high velocity penetration

Clinical manifestationIrregular dyspigmentation at site of skininjury

Amalgam tattoo variant: punctate gray dis-coloration in oral mucosa secondary topenetration of dental amalgam with dentalprocedures; some particles may extrudewithout therapy

Differential diagnosisMelanoma; melanocytic nevus; lentigo;drug-induced pigmentation; exogenousochronosis

TherapyAblation by Q-switched laser; surgical exci-sion; dermabrasion; laser resurfacing;chemical peel

ReferencesFusade T, Toubel G, Grognard C, Mazer JM (2000)

Treatment of gunpowder traumatic tattoo by Q-switched Nd:YAG laser: an unusual adverse effect. Dermatologic Surgery 26(11):1057–1059

Trenaunay syndrome


Trench fever

Synonym(s)5–day fever; quintan fever; shinbone fever;shank fever; His-Werner disease; Wolhyniafever; urban trench fever

DefinitionBlood-borne bacterial infection character-ized by fever, systemic signs and symp-toms, and an eruption occurring at theonset of the disease

PathogenesisCaused by Bartonella quintana, gram nega-tive bacteria introduced to human host bybody louse; inoculation of organism inlouse feces through a skin break or a lousebite


578 Trench foot

Clinical manifestationFever, varying from single episode to recur-rent episodes to persistently elevated bodytemperature for weeks; conjunctivitis; skineruption most commonly occurring duringfirst fever episode; groups of erythematousmacules or papules on abdomen, chest, andback; splenomegaly; hepatomegaly; tachy-cardia

Differential diagnosisBabesiosis; bacillary angiomatosis; crypto-coccosis; Lyme disease; relapsing fever;Rocky Mountain spotted fever; HIV infec-tion; tuberculosis

TherapyDoxycycline; erythromycin; azithromycin

� Bartonellosis

ReferencesOhl ME, Spach DH (2000) Bartonella quintana

and urban trench fever. Clinical Infectious Dis-eases 31(1):131–135

Trench foot

� Immersion foot

Trench mouth

� Acute necrotizing gingivitis

Tretinoin

Trade name(s)Retin-A; Retin A Micro; Avita; Renova


Drug classRetinoid

Mechanism of actionGene transcription after membrane recep-tor binding and intracellular transport; nor-malizes follicular keratinization

Dosage form0.025%, 0.05%, 0.1% cream; 0.04%, 0.1%micro gel; 0.025% gel


Common side effectsCutaneous: scaling, erythema, blistering,photosensitivity


Drug interactionsBenzoyl peroxide; isotretinoin; photosensi-tizing drugs


ReferencesBershad S (2001) Developments in topical retin-

oid therapy for acne. Seminars in Cutaneous Medicine & Surgery 20(3):154–161

Triamcinolone


Trichilemmal cyst

� Pilar cyst


Trichilemmal cyst 579

T

Tretinoin. Dermatologic indications and dosage


Acanthosis nigricans Apply once daily, preferably at bedtime; apply 20–30 minutes after washing and drying skin

Apply once daily, preferably at bedtime; apply 20–30 minutes after washing and drying skin

Acne vulgaris Apply once daily, preferably at bedtime; apply 20–30 minutes after washing and drying skin


Acrokeratoelastoidosis Apply once daily, preferably at bedtime; apply 20–30 minutes after washing and drying skin

Not applicable




Actinic elastosis Apply once daily, preferably at bedtime; apply 20–30 minutes after washing and drying skin

Not applicable

Actinic keratosis Apply twice daily for up to 3 months Apply once daily, preferably at bedtime; apply 20–30 minutes after washing and drying skin

Bowenoid papulosis Apply twice daily for up to 3 months Not applicable

Chloracne Apply once daily, preferably at bedtime; apply 20–30 minutes after washing and drying skin





Favre Racouchot disease


Not applicable

Fox-Fordyce disease Apply once daily, preferably at bedtime; apply 20–30 minutes after washing and drying skin


Hairy tongue Apply twice daily for up to 3 months Apply once daily, preferably at bedtime; apply 20–30 minutes after washing and drying skin



Not applicable

Keratosis pilaris Apply once daily, preferably at bedtime; apply 20–30 minutes after washing and drying skin


Kyrle’s disease Apply once daily, preferably at bedtime; apply 20–30 minutes after washing and drying skin



580 Trichilemmoma

Trichilemmoma

Synonym(s)Tricholemmoma

DefinitionBenign neoplasm with differentiationtoward pilosebaceous follicular epithelium

PathogenesisUnknown

Clinical manifestationAsymptomatic, slow growing papule and/orplaque on face, ear, or upper extremity;small, flesh-colored papules; small plaques,particularly in the nasolabial fold region;with enlargement, thick hyperkeratotic sur-face suggestive of wart

Lamellar ichthyosis Apply once daily, preferably at bedtime; apply 20–30 minutes after washing and drying skin


Melasma Apply once daily, preferably at bedtime; apply 20–30 minutes after washing and drying skin


Nevus comedonicus Apply once daily, preferably at bedtime; apply 20–30 minutes after washing and drying skin


Nevus verrucosus Apply once daily, preferably at bedtime; apply 20–30 minutes after washing and drying skin


Perforating folliculitis Apply once daily, preferably at bedtime; apply 20–30 minutes after washing and drying skin


Photo-aging Apply once daily, preferably at bedtime; apply 20–30 minutes after washing and drying skin

Not applicable

Pomade acne Apply once daily, preferably at bedtime; apply 20–30 minutes after washing and drying skin








Rosacea Apply once daily, preferably at bedtime; apply 20–30 minutes after washing and drying skin


Striae Apply once daily, preferably at bedtime; apply 20–30 minutes after washing and drying skin


Tretinoin. Dermatologic indications and dosage (Continued)



Trichoepithelioma 581

T

Differential diagnosisBasal cell carcinoma; epidermoid cyst; wart;neurilemmoma; trichoepithelioma; tricho-folliculoma; clear cell acanthoma

TherapyShave removal; elliptical excision

ReferencesTellechea O, Reis JP, Baptista AP (1992) Desmo-

plastic trichilemmoma. American Journal of Dermatopathology 14(2):107–114

Trichoblastoma


Trichodiscoma

Synonym(s)Neurofollicular hamartoma

DefinitionHamartomatous proliferation of mesoder-mal component of haarscheibe, slowlyreacting nerve receptor around hair follicle

PathogenesisUnknown

Clinical manifestationSolitary or multiple, discrete, flat-toppedpapules, usually located on central face

Differential diagnosisTrichoepithelioma; trichofolliculoma;angiofibroma; syringoma; basal cell carci-noma; acrochordon


ReferencesNova MP, Zung M, Halperin A (1991) Neurofollic-

ular hamartoma. A clinicopathological study.

American Journal of Dermatopathology 13(5):459–462

Trichoepithelioma

Synonym(s)Trichoblastoma; epithelioma adenoidescysticum; trichoepithelioma papulosummultiplex; sclerosing epithelial hamar-toma; Brooke tumor

DefinitionBenign adnexal tumor with differentiationtoward hair follicle epithelium

PathogenesisAutosomal dominant familial form relatedto a mutation in tumor suppressor gene,located on 9q21

Clinical manifestationRound, skin-colored, firm papule or nod-ule, located mainly on nasolabial folds,nose, forehead, upper lip, and scalp; occa-sional lesions on neck and upper trunk;rare ulceration; multiple lesions in familialform, usually on nasolabial folds; solitarygiant trichoepithelioma: large, polypoidtumor, usually in the lower trunk or in glu-teal area

Differential diagnosisBasal cell epithelioma; colloid milium;cylindroma; angiofibroma; milium; pilar

Trichoepithelioma. Multiple flesh-colored papules in the central facial area


582 Trichoepithelioma papulosum multiplex

cyst; syringoma; trichilemmoma; micro-cystic adnexal carcinoma

TherapySolitary tumor: surgical excision or shaveremovalMultiple tumors: CO2 laser ablation; der-mabrasion

ReferencesSmith KJ, Skelton HG, Holland T (1992). Recent

advances and controversies concerning adnex-al neoplasms. Dermatologic Clinics 10(1):117–160

Trichoepithelioma papulosum multiplex


Trichofolliculoma

Synonym(s)Folliculoma; hair follicle nevus

DefinitionHamartoma of follicular epithelium, typi-cally occurring on the face

PathogenesisMay be abortive differentiation of pluripo-tent skin cells towards hair follicles

Clinical manifestationSingle, flesh-colored or whitish papule, typ-ically on face, most frequently around thenose; central pore or black dot, sometimesdraining sebaceous-like material; tuft ofwhite hair sometimes emerges from centralpore

Differential diagnosisBasal cell epithelioma; colloid milium;cylindroma; angiofibroma; milium; pilar

cyst; syringoma; trichilemmoma; micro-cystic adnexal carcinoma; trichoepitheli-oma; vellus hair cyst


ReferencesLabandeira J, Peteiro C, Toribio J (1996) Hair fol-

licle nevus: case report and review. American Journal of Dermatopathology 18(1):90-93

Tricholemmoma

� Trichilemmoma

Trichomalacia

DefinitionDamage to anagen hair root by repeatedplucking or other injury, characterized bydeformed and twisted bulb, seen mainlywith trichotillomania

ReferencesWalsh KH, McDougle CJ (2001) Trichotillomania.

presentation, etiology, diagnosis and therapy. American Journal of Clinical Dermatology 2(5):327–333

Trichomatricoma

� Pilomatricoma

Trichomatrioma

� Pilomatricoma


Trichorrhexis nodosa 583

T

Trichomycosis axillaris and pubis

Synonym(s)Trichomycosis nodosa; trichomycosis nod-ularis

DefinitionSuperficial bacterial colonization of theaxillary hair shafts, characterized by granu-lar concretions adhering to hair shaft

PathogenesisCaused by several species of the gram-posi-tive diphtheroid Corynebacterium over-growth on hair shafts in moist regions ofthe body

Clinical manifestationSeen more often in tropical climates; some-times associated with hyperhidrosis; con-cretions encircling hair shaft, giving itbeaded appearance; most common on thecentral portion of axillary hair (trichomy-cosis axillaris) or inguinal region, often onscrotum (trichomycosis pubis); red, black,or yellow concretions firmly adhering tohair shaft; yellow color sometimes stainsclothes yellow, black, and red

Differential diagnosisPediculosis; piedra; hair casts; soap or deo-dorant remnants

TherapyShaving of affected hair�; use of antiperspi-rants to prevent recurrence

ReferencesO'Dell ML (1998) Skin and wound infections: an

overview. American Family Physician 57(10):2424–2432

Trichomycosis nodosa

� Trichomycosis axillaris and pubis

Trichomycosis nodularis

� Piedra� Trichomycosis axillaris and pubis

Trichophytosis barbae

� Sycosis barbae

Trichopoliodystrophy

� Menkes kinky hair syndrome

Trichorrhexis invaginata

DefinitionHair fibers having the shape of bamboo;fibers with focal nodules making themresemble a bamboo shoot; focal defects inthe hair fiber, with development of a cupand ball shape; seen in Netherton’s syn-drome

ReferencesRogers M (1996) Hair shaft abnormalities: Part II.


Trichorrhexis nodosa

DefinitionDefect in the hair shaft characterized bythickening or weak points (nodes) causingthe hair to break easily; precipitated byenvironmental insults in disorders such asargininosuccinic aciduria, Menkes' kinkyhair syndrome, Netherton's syndrome,hypothyroidism, or trichothiodystrophy


584 Trichosporosis

ReferencesRogers M (1995) Hair shaft abnormalities: Part I.


Trichosporosis

� Piedra

Trichostasis spinulosa

Synonym(s)None

DefinitionDark follicular papules, caused by multiplevellus hairs imbedded in follicular orifice

PathogenesisResults from successive production andretention of vellus telogen club hairs fromsingle hair matrix in single follicle

Clinical manifestationDark, follicular plugs or papules, some-times with tufts or spines of fine hair protu-ding; most common on nose and uppertrunk

Differential diagnosisComedonal acne; lichen spinulosus;retained dirt; keratosis pilaris

TherapyDepilatory wax or adhesive strips; drainagewith comedone extractor

ReferencesHarford RR, Cobb MW, Miller ML (1996) Trichos-

tasis spinulosa: a clinical simulant of acne open comedones. Pediatric Dermatology 13(6):490–492

Trichothiodystrophy

� Tay syndrome

Trichothiodystrophy with congenital ichthyosis

� Tay syndrome

Trichotillomania

Synonym(s)Chronic hair pulling; morbid hair pulling;compulsive hair pulling

DefinitionAlopecia caused by compulsive pulling and/or twisting of the hair until it breaks off

PathogenesisImpulse control disorder, often with under-lying emotional problem; become habitualonce behavior is established, regardless ofinitial emotional problem

Trichotillomania. Alopecic plaque with broken hairs in the scalp


Trimox 585

T

Clinical manifestationIncomplete nonscarring alopecia, in rela-tively localized sites; geometric shapes ofinvolved area, with broken hair; occursmost frequently in scalp, but sometimesinvolves eyebrows or eyelashes

Differential diagnosisAlopecia areata; tinea capitis; androgeneticalopecia; syphilis; lupus erythematosus;monilethrix; traction alopecia; pili torti;temporal triangular alopecia

TherapySelective serotonin reuptake inhibitors inpatients unable to control impulse afterunderstanding nature of disorder

ReferencesHautmann G, Hercogova J, Lotti T (2002) Tri-

chotillomania. Journal of the American Acade-my of Dermatology 46(6):807–821

Trichrome vitiligo

� Vitiligo

Triglyceride storage disease


Trimethoprim-sulfamethoxazole

Trade name(s)Bactrim; Septra



Mechanism of actionInhibition of enzymes involved in bacterialtetrahydrofolic acid synthesis

Dosage formDS capsule


Common side effectsCutaneous: urticaria or other vascular reac-tion, photosensitivityGastrointestinal: anorexia, nausea, vomit-ing, diarrheaNeurologic: dizziness

Serious side effectsBone marrow: aplastic anemia, agranulocy-tosisCutaneous: Stevens-Johnson syndrome,toxic epidermal necrolysisGastrointestinal: hepatitis, hepatic necro-sis, pseudomembranous colitisRenal: interstitial nephritis

Drug interactionsOral contraceptives; dapsone; MAO inhibi-tors; metformin; methotrexate; phenytoin;probenecid; procainamide; sulfonylureas;warfarin

Contraindications/precautionsHypersensitivity to drug class or compo-nent; folate deficiency; G6PD deficiency

References Smilack JD (1999) Trimethoprim-sulfamethoxa-

zole. Mayo Clinic Proceedings 74(7):730–734

Trimox

� Amoxicillin


586 Tropical anhidrosis

Tropical anhidrosis

� Miliaria

Tropical anhidrotic asthenia

� Acquired generalized anhidrosis

Tropical jungle foot

� Immersion foot


Synonym(s)Vincent’s ulcer; tropical sloughingphagedena; ulcus tropicum

DefinitionAcute, painful, destructive skin ulcerationoccurring in presence of fusiform bacilliand spirochetes

PathogenesisMultiple contributing factors, includingprotein deficiency, presence of fusiformbacilli and spirochetes, and minor traumato affected site

Clinical manifestationPapule or vesicle at site of minor trauma,often on lower extremity; rapid evolution ofnecrotic, purulent, putrid ulceration oftendown to fascia, tendon, and bone; chronicstage with indolent, non-purulent ulcera-tion

Differential diagnosisLeishmaniasis; bacterial pyoderma; pyo-derma gangrenosum; cutaneous diphthe-ria; gummatous syphilis; yaws; leprosy;chromomycosis; squamous cell carcinoma;venous stasis ulcer; atypical mycobacterialinfection; venomous sting or bite

TherapyAcute stage: Benzathine penicillin G�; tet-racycline; metronidazole: 400 mg PO 3times daily until healingChronic stage: no specific antibiotic therapy

ReferencesRobinson DC, Adriaans B, Hay RJ, Yesudian P

(1988) The clinical and epidemiologic features of tropical ulcer (tropical phagedenic ulcer).

Trimethoprim-sulfamethoxazole. Dermatologic indications and dosage


Granuloma inguinale DS capsule twice daily for at least 3 weeks

Not established

Melioidosis DS capsule twice daily until ulceration heals

Not established

Mycetoma DS capsule twice daily until ulceration heals

Not established


DS capsule PO twice daily for 4–6 weeks after clincial resolution

Not established

Nocardiosis DS capsule twice daily for at least 3 weeks

Not established


DS capsule twice daily for 2–3 years Not established


Tuberous sclerosis 587

T

International Journal of Dermatology 27(1):49–53

Tropical sloughing phagedena

� Tropical phagedenic ulcer

Tsutsugamushi disease

� Scrub typhus

Tsutsugamushi fever

� Scrub typhus

Tuberculosis, cutaneous


Tuberculosis cutis orificialis


Tuberculosis cutis verrucosa


Tuberculosis of skin


Tuberculosis verrucosa cutis


Tuberculous chancre


Tuberculous gumma


Tuberous sclerosis

Synonym(s)Epiloia; Bourneville disease; tuberous scle-rosis complex

DefinitionHereditary disorder characterized byhamartomas in multiple organs

PathogenesisAutosomal dominant trait; mutations ofgenes coding for hamartin and tuberin,involved in the regulation of cell prolifera-tion and differentiation (hamartin) andtumor suppression (tuberin)

Clinical manifestationSkin lesions: angiofibromas (adenomasebaceum) often in nasolabial folds and oncheeks and chin; periungual fibromas(Koenen tumors); connective tissue nevus(Shagreen patch), presenting as flesh-colored, soft plaque in the lumbosacralarea; ash leaf-shaped macules on trunk orlimb; guttate leukoderma; café au lait mac-ules; poliosisNeurologic changes: tuberosclerotic nod-ules of glial proliferation in cerebral cortex,


588 Tuberous sclerosis complex

basal ganglia, and ventricular walls;number of tubers appears to correlate withclinical disease severity; epilepsy; mentalretardationOther features noted: schizophrenia; autis-tic behavior; and attention-deficit hyperac-tivity disorderMiscellaneous findings: cardiac rhabdomy-omas; aortic aneurysm; renal angiomyol-ipoma and renal cysts; pulmonary lym-phangiomatosis with cyst formation;microhamartomatous polyps in bone cysts;pituitary adrenal dysfunction; thyroid dis-orders; premature puberty; diffuse cutane-ous reticulohistiocytosis; gigantism

Differential diagnosisAcne; connective tissue nevus; nevus ane-micus; vitiligo; warts; trichoepithelioma;syringoma; rosacea

TherapyPulsed dye or CO2 laser ablation or derma-brasion for facial angiofibromas; CO2 laservaporization for periungual fibromas

ReferencesHarris-Stith R, Elston DM (2002) Tuberous scle-

rosis. Cutis 69(2):103–109

Tuberous sclerosis complex


Tuberous xanthoma

� Xanthoma

Tufted angioma

Synonym(s)Nakagawa’s angioma; Nakagawa's angio-blastoma; progressive capillary hemangi-

oma; acquired tufted angioma;angioblastoma

DefinitionVascular skin tumor, characterized by slowangiomatous proliferation and a distinctivehistologic presentation

PathogenesisOccasional occurrence within port winestains

Clinical manifestationSolitary or multifocal, sometimes painful,purplish-red to red-brown patch or plaquepredominantly appearing on upper trunk,neck, or shoulders; less commonly occur-ring on face, scalp, or proximal extremities

Differential diagnosisCapillary hemangioma; Kaposi’s sarcoma;kaposiform hemangioendothelioma;hemangiopericytoma; pyogenic granu-loma; endovascular papillary angioen-dothelioma; melanoma

TherapySurgical excision; pulse dye laser ablation

ReferencesOkada E, Tamura A, Ishikawa O, Miyachi Y (2000)

Tufted angioma (angioblastoma): case report and review of 41 cases in the Japanese litera-ture. Clinical & Experimental Dermatology 25(8):627–630

Tularemia

Synonym(s)Rabbit fever; deer-fly fever; wild hare dis-ease; water-rat trapper’s disease; marketmen’s disease

DefinitionAcute infectious zoonosis, characterized byskin eruption and/or ulceration, lymphade-nopathy, and variable systemic signs andsymptoms


Tungiasis 589

T

PathogenesisCaused by aerobic gram-negative pleomor-phic bacillus Francisella tularensis, afterintroduction of bacillus by inhalation,intradermal injection, or oral ingestion;rabbits and ticks (especially Dermatocen-tor and Amblyomma species) most com-mon vectors

Clinical manifestationUlceroglandular variant: organism usuallygaining entry via scratch or abrasion; ulcerat the site of entry begins as tender papuleand eventually ulcerates; sharply demar-cated border with a yellowish exudate; baseof the ulcer with yellow exudate becomesblack; regional lymphadenopathyGlandular variant: similar to ulceroglandu-lar form except for absence of skin lesionOculoglandular variant: organism entersvia the conjunctivae after inoculation fromeither splashing of blood or rubbing of eyesafter contact with infectious materials; uni-lateral, painful, purulent conjunctivitis withpreauricular or cervical lymphadenopathyOropharyngeal variant: occurs after eatingpoorly cooked rabbit meat; sore throat;abdominal pain; nausea; vomiting;diarrhea; and; occasional gastrointestinalbleedingPneumonic variant: occurring after inhala-tion of organism; pneumonia also some-times occurs after hematogenous spread inpatients with ulceroglandular tularemia ortyphoidal tularemia; dry cough; dyspnea;and pleuritic-type chest painTyphoidal (septicemic) variant: representsbacteremia; fever; chills; myalgias; malaise;weight loss, often with subsequent pneumo-nia

Differential diagnosisAnthrax; orf; milker’s nodule; foreign bodygranuloma; Q fever; Rocky Mountain spot-ted fever; Lyme disease; Majocchi’s granu-loma; sporotrichosis; coccidioidomycosis;North American blastomycosis; plague;brucellosis; diphtheria; bacterial endocardi-tis; legionella infection; malaria; mononucl-eosis; syphilis; rat bite fever; atypical myco-bacterial infection

TherapyStreptomycin: adult dose: 1–2 gm IM, giventwice daily for 7–14 days or until patient isafebrile for 5–7 days; pediatric dose: 20–40mg per kg per day IM given twice daily for7–14 days or until patient is afebrile for 5–7daysw; doxycycline

ReferencesChoi E (2002) Tularemia and Q fever. Medical

Clinics of North America 86(2):393–416

Tungiasis

Synonym(s)None

DefinitionInfestation by burrowing human flea

PathogenesisCaused by infestation with the burrowingflea, Tunga penetrans, common in CentralAmerica, South America, India, and tropi-cal Africa; major risk factor: failure to wearshoes when walking in sand in an area withactive infestation; upon contact, fleasinvade unprotected skin

Clinical manifestationCommon areas of involvement: plantarfoot, intertriginous regions of the toes, andperiungual regions; pruritic white papulewith central black dotMore advanced infestation: crusted ery-thematous papules, painful pruritic nod-ules, crateriform lesions, and secondaryinfection including lymphangitis and septi-cemia

Differential diagnosisInsect bite reaction; scabies; cercarial der-matitis; tick bite; myiasis; fire ant sting;creeping eruption; dracunculiasis

TherapySurgical extirpation of the parasite usingsterile needle or curette�


590 Turban tumor

ReferencesFein H, Naseem S, Witte DP, Garcia VF, Lucky A,

Staat MA (2001) Tungiasis in North America: a report of 2 cases in internationally adopted children. Journal of Pediatrics 139(5):744–746

Turban tumor

� Cylindroma

Turner Kieser syndrome


Turner phenotype syndrome


Turner syndrome

Synonym(s)Bonnevie-Ullrich syndrome; gonadal dys-genesis

DefinitionDisorder in women caused by a chromo-somal defect, producing impaired sexualdevelopment, infertility, and multiple othercongenital defects

PathogenesisResults from lack of second SHOX gene onX chromosome; many features, includingthe short stature

Clinical manifestationShort stature; signs of ovarian failure; hypo-plastic or hyperconvex nails; many nevocel-

lular nevi; cutis laxa; webbed neck; skeletalanomalies including cubitus valgus, scolio-sis, short fourth metacarpal or metatarsalbone, shield chest, hip dislocation; eyechanges including ptosis, strabismus,amblyopia and cataracts; gastrointestinalbleeding

Differential diagnosisNoonan’s syndrome; gonadal dysgenesis;autoimmune thyroiditis; XY gonadal agene-sis syndrome


ReferencesCunniff C (2002) Turner syndrome. Adolescent

Medicine State of the Art Reviews 13(2):359–366

Turner-like syndrome


Twenty nail dystrophy

Synonym(s)Twenty nail dystrophy of childhood;trachyonychia

DefinitionAcquired nail abnormality characterized byrough linear ridges on many but not neces-sarily all twenty nails of the fingers and toes

PathogenesisMany cases with no known cause; someassociated with alopecia areata, psoriasis,lichen planus, atopy, ichthyosis, or otherinflammatory dermatoses

Clinical manifestationRough linear edges of nail plates; opales-cent and frequently brittle nail plates thatsplit at free margin; more common in chil-


Typhus 591

T

dren, with tendency for improvement withincreased age

Differential diagnosisOnychomycosis; lichen planus; psoriasis;onychophagia; traumatic nail dystrophy


ReferencesTosti A, Bardazzi F, Piraccini BM, Fanti PA (1994)

Idiopathic trachyonychia (twenty-nail dystro-phy): a pathological study of 23 patients. British Journal of Dermatology 131(6):866–872

Twenty-nail dystrophy of childhood

� Twenty nail dystrophy

Tylosis

Synonym(s)Keratosis palmaris et plantaris with carci-noma of the esophagus; Howell-Evans syn-drome

DefinitionFamilial hyperkeratosis of the palms andsoles associated with carcinoma of theesophagus

PathogenesisAutosomal dominant gene; tylosis esopha-geal cancer gene (TOC) localized to chro-mosome 17q25

Clinical manifestationFocal palmoplantar keratoderma begin-ning by age 5–15 years; variable oral leukok-eratosis; follicular keratosis; increased sus-ceptibility to carcinoma of esophagus

Differential diagnosisTyrosinemia type II; pachyonycia congen-ita; focal palmoplantar and oral mucosahyperkeratosis; acrokeratoelastoidosis;focal acral hyperkeratosis; acrokeratois ofBazex; arsenical keratosis

TherapyAlpha hydroxy acids; emollients; urea

ReferencesCohen PR, Kurzrock R (1995) Miscellaneous gen-

odermatoses: Beckwith-Wiedemann syn-drome, Birt-Hogg-Dube syndrome, familial atypical multiple mole melanoma syndrome, hereditary tylosis, incontinentia pigmenti, and supernumerary nipples. Dermatologic Clinics 13(1):211–229

Type II histiocytosis


Typhoid fever

� Salmonellosis

Typhus

Synonym(s)Rickettsemia

DefinitionGroup of infectious diseases caused by rick-ettsial organisms and producing acutefebrile illness

ReferencesCowan G (2000) Rickettsial diseases: the typhus

group of fevers – a review. Postgraduate Medi-cal Journal 76(895):269–272


592 Typus degenerativus amstelodamensis

Typus degenerativus amstelodamensis


Tyrosinemia II

Synonym(s)Richner-Hanhart syndrome; Hanhart-Rich-ner syndrome; tyrosinosis; keratosispalmo-plantaris circumscripta

DefinitionHereditary disease characterized by tyro-sinemia, palmar and plantar erosion, kera-titis, and occasional mental retardation

PathogenesisDeficiency of hepatic tyrosine aminotrans-ferase, leading to elevated levels of tyro-sine, which crystalizes in tissues and causesinflammatory response

Clinical manifestationSkin findings: painful erosions of the palmsand soles, which become crusted and then

hyperkeratotic; hyperkeratosis of thetongue.Ocular findings: tearing and photophobia;corneal ulcerations and subsequent scar-ring.Neurologic findings: mental retardation;self-mutilating behavior; fine coordinationdisturbances

Differential diagnosisOther forms of focal palmo-plantar kerato-derma, such as Wachter syndrome andHowel-Evans syndrome; epidermolysis bul-losa; Spanlang-Tappeiner syndrome

TherapyLow tyrosine, low phenylalanine diet, suchas Mead Johnson 3200 AB�; acitretin

ReferencesRabinowitz LG, Williams LR, Anderson CE, Maz-

ur A, Kaplan P (1995) Painful keratoderma and photophobia: hallmarks of tyrosinemia type II. Journal of Pediatrics 126(2):266–269

Tyrosinosis

� Tyrosinemia II


U

Ulcus tropicum

�


Ulerythema

�

Ulerythema ophryogenes

Ulerythema acneiforme

�



Synonym(s)

Ulerythema

;

keratosis pilaris rubra atrophi-cans faciei

;

folliculitis ulerythema reticu-lata

;

honeycomb atrophy

;

atrophodermavermiculatum

;

keratosis pilaris atrophicans

Definition

Disorder characterized by inflammatorykeratotic facial papules with scarring, atro-phy, and alopecia

Pathogenesis

May be subset of keratosis pilaris


Erythema with follicular hyperkeratosis oncheeks and lateral aspects of eyebrows;occasional scalp involvement; generalizedfacial erythema with scattered open andclosed comedones and milia; hyperkera-totic follicular papules with surroundingerythema evolving into coalescent follicu-lar depressions in a honeycombed pattern;improvement with age


Keratosis pilaris; acne vulgaris; folliculitis;rosacea; lupus erythematosus; pityriasisrubra pilaris; constitutive flushing

Therapy

585-nm pulse dye laser ablation; alphahydroxy acids

�


References

Clark SM, Mills CM, Lanigan SW (2000) Treat-ment of keratosis pilaris atrophicans with the pulsed tunable dye laser. Journal of Cutaneous Laser Therapy 2(3):151–156

Ullrich-Noonan syndrome

�

Noonan’s syndrome


594 Uncombable hair syndrome

Uncombable hair syndrome

Synonym(s)

Spun glass hair

;

cheveux incoiffables

;

pilitrianguli et canaliculi

Definition

Hereditary disorder characterized by dry,brittle, hypopigmented, spangled scalp hair

Pathogenesis

Autosomal dominant trait; hair fiber inflex-ible, making it difficult to lay flat againstthe scalp


Most frequently develops shortly after birthbut possibly any time until puberty; slow-growing scalp hair, with little or no pig-ment, easily pulled out; very dry; some-times brittle; spangled appearance; eye-brow and eyelash hairs usually normal butsometimes sparse; nails sometimes short,brittle, and easy to split; teeth aberrationssuch as enamel defects; possibility of spon-taneous recovery with advancing age


Loose anagen hair syndrome; monilethrix;pili torti; Marie-Unna syndrome; progeria;Menke disease

Therapy


References

Hicks J, Metry DW, Barrish J, Levy M (2001) Un-combable hair (cheveux incoiffables, pili trian-guli et canaliculi) syndrome: brief review and role of scanning electron microscopy in diag-nosis. Ultrastructural Pathology 25(2):99–103

Universal acquired melanosis

Synonym(s)

Carbon baby

Definition

Progressive generalized hyperpigmentation

Pathogenesis

Increased pigmentation secondary toincreased number of melanocytes andincreased melanization in the epidermis


Onset in the first few months of life; slowlyincreasing pigmentation of the skin andmucous membranes


Normal racial pigmentation; Addison’s dis-ease; bronze baby; Schilder’s disease

Therapy

None

References

Ruiz-Maldonado R, Tamayo L, Fernandez-Diez J (1978) Universal acquired melanosis. The car-bon baby. Archives of Dermatology 114(5):775–778

Unna-Thost palmoplantar keratoderma

Synonym(s)

Diffuse nonepidermolytic palmoplantarkeratoderma

;

Thost-Unna disease

;

palmoplantar keratoderma diffusa circum-scripta

;

congenital keratoderma of thepalms and soles

;

hereditary palmo-plantarkeratoderma

;

hyperkeratosis palmaris etplantaris

;

ichthyosis palmaris et plantaris

Definition

Hereditary keratoderma of the palms andsoles, characterized by thick plaques overpalms and soles

Pathogenesis

Autosomal dominant trait; linkage to typeII keratin locus on 12q11–13


Urea, topical 595

U


Keratotic lesions confined to palms andsoles; thick, horny, hard, yellowish plaqueswith waxy smooth surfaces; plaques some-times pitted and verrucous, surrounded byerythematous halos; occasional cornealopacites; pili torti, sensorineural hearingloss; hypohidrosis; dental abnormalities


Mal de Meleda; Papillon-Lefèvre syndrome;hereditary epidermolytic palmoplantar ker-atoderma; Vohwinkel syndrome; Richner-Hanhart syndrome; progressive kerato-derma; punctate keratoderma; pityriasisrubra pilaris; xerosis

Therapy

Alpha hydroxy acids; urea; keratolyticagents such as salicylic acid 6 % gel; propyl-ene glycol 60 %

References

Zemtsov A, Veitschegger M (1993) Keratodermas. International Journal of Dermatology 32(7):493–498

Urbach-Wiethe disease

�

Lipoid proteinosis

Urban trench fever

�

Bartonellosis

�

Trench fever

Urea, topical

Trade name(s)

Aquacare; Neutraplus; Carmol; Ultramide;Ureacin

Generic available

Yes

Drug class

Emollient; keratolytic agent

Mechanism of action

Hydrophilic property allows for waterretention in stratum corneum; protein sol-vent and denaturant; chemical hygroscopickeratolysis

Dosage form

10%, 20%, 40% cream; 25% lotion


See table

Common side effects

Cutaneous:

burning sensation, stinging,irritation


None

Drug interactions

None



Urea, topical. Dermatologic indications and dosage


Ichthyosis Apply twice daily Apply twice daily

Keratoderma Apply twice daily Apply twice daily


Xerosis Apply twice daily Apply twice daily


596 Uremic gangrene syndrome

References

Swanbeck G (1992) Urea in the treatment of dry skin. Acta Dermato-Venereologica (Suppl) 177:7–8

Uremic gangrene syndrome

�

Calciphylaxis

Uremic necrosis

�

Calciphylaxis

Uremic pruritus

Synonym(s)

None

Definition

Pruritus occurring in patients with chronicrenal failure

Pathogenesis

May involve unidentified pruritogenicsubstances accumulating in dialysispatient as a result of molecular size; othertheories: xerosis; hyperparathyroidism;hypercalcemia; hyperphosphatemia; ele-vated plasma histamine levels; uremicneuropathy


Generalized or localized paroxysmal pruri-tus, most commonly occurring on forearmand back


Xerosis; atopic dermatitis; scabies; drug-induced pruritus; hyperthyroidism; hyper-parathyroidism; psychogenic pruritus

Therapy

UVB phototherapy

�

; naltrexone: 50 mg POdaily; cholestyramine: 4 gm PO twice daily;activated charcoal: 6 gm PO daily dividedinto 4–6 doses; antihistamines, first genera-tion; emollients; acupuncture

References

Urbonas A, Schwartz RA, Szepietowski JC (2001) Uremic pruritus–an update. American Journal of Nephrology 21(5):343–350

Urticaria

Synonym(s)

Hives

Definition

Hypersensitivity reaction, causing tran-sient erythema and edema

Pathogenesis

Allergic and non-allergic mechanisms oper-ative; final common pathway histamine andother mediator release from mast cells; inallergic reactions, adjacent IgE molecules,bound to the surface of mast cells by the IgEreceptors, cross-linked by allergens, lead tothe release of histamine and other media-tors; most commonly related to reactions tomedications or infections; sometimesrelated to foods, food dyes and preserva-tives, rheumatic disorders, neoplastic dis-eases


Transient, pruritic, edematous, pink or redpapules or plaques (wheals) of variable sizeand shape, with surrounding erythemaAngioedema variant: ill-defined, subcuta-neous, edematous plaques, with associatedpruritus, pain, or burning sensation inlesionsPhysical urticaria (dermatographism): urti-carial wheal at site of light stroking or rub-bing; may occur with concomitant chronic


Urticarial vasculitis 597

U

idiopathic urticaria; pressure-induced urti-caria; delayed response to pressure appliedto skinCold urticaria: wheal at site of cold applica-tion; may occur with rapid temperaturechange, without extremes of coldSolar urticaria: wheals after brief exposureto sunlightCholinergic urticaria: small wheals trig-gered by heat, exercise, or emotional stressExercise-induced urticaria: wheals appear-ing after vigorous exerciseAquagenic urticaria: wheals appearing afterexposure to water


Urticarial vasculitis; erythema multiforme;insect bite reaction; mastocytosis; bullouspemphigoid; pruritic urticarial papules andplaques of pregnancy; Melkersson-Rosenthal syndrome

Therapy

Antihistamines, first generation; antihista-mines, second generation; severely sympto-matic, recalcitrant disease: prednisone;nifedipine: 10 mg PO 2–3 times daily; cyclo-sporine; dapsone

References

Grattan CE, Sabroe RA, Greaves MW (2002) Chronic urticaria. Journal of the American Academy of Dermatology 46(5):645–657

Urticaria neonatorum

�

Erythema toxicum

Urticaria pigmentosa

�

Mastocytosis

Urticarial vasculitis

Synonym(s)


Definition

Urticaria-like eruption with histologic find-ings of vasculitis

Pathogenesis

Antigen-antibody complexes deposited inthe vascular lumina, resulting in comple-ment activation and chemotaxis of neu-trophils; cells release proteolytic enzymes,such as collagenase and elastase, resultingin damage to the vascular lumina


Erythematous wheals, accompanied by apainful or burning sensation, which remainfor several days; as lesions evolve, purpuramay appear; lesions may resolve withpostinflammatory pigmentation; associ-ated photosensitivity, lymphadenopathy,arthralgia, angioedema, fever, abdominalpain, dyspnea, and pleural and pericardialeffusionsMain identifiable causes: drug induced,such as angiotensin-converting enzymeinhibitors, penicillin, sulfonamides, fluoxet-ine, and thiazides; rheumatic diseases, suchas lupus erythematosus and Sjögren syn-drome; viral diseases, such as hepatitis B,hepatitis C, and infectious mononucleosis;hypocomplementemia occurs in patientswith associated systemic diseases, such assystemic lupus erythematosus; regardless ofcause, disease tends to run chronic cours


Urticaria; allergic cutaneous vasculitis; ery-thema multiforme

Therapy

Antihistamines, second generation; recalci-trant disease: colchicine; hydroxychloro-quine; dapsone; systemic disease: pred-nisone


598 Uveoencephalitis

References

Black AK (1999) Urticarial vasculitis. Clinics in Dermatology 17(5):565–569

Uveoencephalitis

�


Uveomeningoencephalitic syndrome

�



V

Valacyclovir

Trade name(s)

Valtrex

Generic available

No

Drug class

Anti-viral

Mechanism of action

DNA polymerase inhibition

Dosage form

500 mg, 1000 mg tablet


See table

Common side effects

Gastrointestinal:

nausea, vomiting

Neurologic:

headache


Bone marrow:

suppression

Gastrointestinal:

hepatitis

Neurologic:

seizures, encephalopathy, coma

Drug interactions

Aminoglycosides; carboplatin; cidofovir;cisplatin; glyburide; metformin; mycophe-nolate mofetil; probenecid; nephrotoxicagents

Other interactions

None


Hypersensitivity to drug class or compo-nent; elderly patients or those with renalfailure may need lower dose

Valacyclovir. Dermatologic indications and dosage


Herpes simplex virus infection, first episode

1000 mg PO twice daily for 10 days Not established


500 mg-1000 mg PO daily for up to 1 year

Not established

Herpes simplex virus infection, recurrent episode

2000 mg PO twice daily for 1 day Not established

Herpes zoster 1000 mg PO 3 times daily for 7 days Not established

Varicella 1000 mg PO 3 times daily for 7 days Not established


600 Valley fever

References

Baker DA (2002) Valacyclovir in the treatment of genital herpes and herpes zoster. Expert Opin-ion on Pharmacotherapy 3(1):51–58

Valley fever

�

Coccidioidomycosis

Van Buren’s disease

�


Varicella

Synonym(s)

Chickenpox

;

primary varicella

Definition

Exanthem caused by the varicella zostervirus

Pathogenesis

Acquired by the inhalation of airborne res-piratory droplets containing virus from aninfected host; viremia disseminates thevirus to the skin; transmission also occursthrough direct contact with virus-contain-ing cutaneous vesicles


Rash, malaise, and low-grade fever at theonset; small, red macules appearing on thescalp, face, trunk, and proximal limbs, withprogression to pruritic papules, vesicles,and pustules; central umbilication andcrust formation as lesions evolve; new cropsof lesions over a few days; infectious for 1–2days prior to the development of rash andfor 4–5 days afterwards; healing withoutscarring, except with excoriation or second-ary bacterial superinfection


Herpes simplex virus infection; drug erup-tion; other viral exanthem; bullous pemphi-goid; dermatitis herpetiformis; erythemamultiforme; pityriasis lichenoides et vari-oliformis acuta; congenital syphilis

Therapy

Immunocompetent adult population: vala-cyclovirImmunocompromised population: intrave-nous acyclovir

�

Highly susceptible, virus-exposed immuno-suppressed populations: varicella-zosterimmune globulin [VZIG]

�

Healthy children: avoidance of use of sali-cylates; calamine lotion, oatmeal baths forpruritus; antihistamines, first generation

References

McCrary ML, Severson J, Tyring SK (1999) Vari-cella zoster virus. Journal of the American Academy of Dermatology 41(1):1–14

Varicose and telangiectatic leg veins

Synonym(s)

Broken capillaries

;

varicosities

;

venectasia

;

varicose veins

;

spider veins

;

swollen veins

Definition

Surface manifestations of an underlyingvenous insufficiency syndrome, character-ized by dilated and tortuous vascular chan-nels on the leg

Pathogenesis

Dilatation of normal veins under the influ-ence of increased venous pressure, mostoften resulting from venous insufficiencydue to valve incompetence in the deep orsuperficial veins; increased venous pres-sure from outflow obstruction, either fromintravascular thrombosis or from extrinsiccompression; changes during pregnancy


Variegate porphyria 601

V

most often caused by hormonal changesrendering vein wall and the valves more pli-able; genetic component to primary valvu-lar failure susceptibility


Visible distension of superficial veins,mostly along the course of greater saphen-ous vein on leg and over medial thigh;sometimes associated with acute varicosecomplications, including variceal bleeding,stasis dermatitis, thrombophlebitis, celluli-tis, and ulceration


Thrombophlebitis; cellulitis; Osler-Weber-Rendu syndrome; stasis dermatitis

Therapy

Small or superficial vein disease: supporthose; intermittent leg elevation; weight loss;chemical sclerosis (sclerotherapy); transcu-taneous laser therapy; intense-pulsed-light(IPL) therapyLarge and deep vein disease: ligation ofsaphenofemoral junction with vein strip-ping; phlebectomy; endovenous radiofre-quency thermal ablation; endovenous laserthermal ablation

References

Weksberg F (1999) Leg vein evaluation and thera-py. Journal of Cutaneous Medicine & Surgery 3 Suppl 4:S43–8

Varicose veins

�


Varicosities

�


Variegate dermatitis

�

Large plaque parapsoriasis

Variegate porphyria

Synonym(s)

Porphyria variegata

;

South African porphy-ria

;

protocoproporphyria

;

mixed porphyria

Definition

Hereditary disorder of porphyrin metabo-lism, characterized by photosensitivity andneurologic dysfunction

Pathogenesis

Autosomal dominant trait; gene mutationencoding defective protoporphyrinogenoxidase; trigger factors: certain drugs, hor-monal fluctuations, carbohydrate restric-tion, infections


Skin manifestations: photosensitivity;mechanical fragility; non-inflammatoryvesicles and bullae, most commonly overdorsum of hands; scarring of sun-exposedskin; hypertrichosis; hyperpigmentationGastrointestinal manifestations: abdominalpain; nausea and vomitingNeurologic manifestations: confusion; diso-rientation; agitation; psychotic behavior;seizures; coma; peripheral neuropathy caus-ing paresthesias, and/or paralysis; auto-nomic neuropathy


Porphyria cutanea tarda; hereditary copro-porphyria; erythropoietic protoporphyria;acute intermittent porphyria; lupus ery-thematosus; polymorphous light eruption;epidermolysis bullosa; epidermolysis bul-losa acquisita; pseudoporphyria; drug-induced photosensitivity


602 Variola

Therapy

Acute attack management: panhematin – 3–5 mg per kg IV 1–2 times daily for 3–4days

�

; strict avoidance of triggers, such asextreme carbohydrate-restricted dieting,certain medications, alcohol, and smoking

References

Lim HW, Cohen JL (1999) The cutaneous porphy-rias. Seminars in Cutaneous Medicine & Sur-gery 18(4):285–292

Variola

Synonym(s)

Smallpox

Definition

Viral infection causing widespread cutane-ous vesicular eruption and serious sys-temic illness

Pathogenesis

Caused by infection with variola virus,spread via the respiratory route; major roleof cell-mediated immunity in controllingdisease; virus-specific cytotoxic T cellssometimes limit viral spread


7–17 day incubation, followed by prodromeof fever, headache, pharyngitis, backache,nausea, vomiting, and feeling of generaldebility; oral mucous membrane enan-them; skin eruption begins with small, redmacules on face and then spreads toextremities and trunk; lesions evolve intofirm papules, then vesiculate, develop intopustules, and coalesce; by day 17, pustulesform crusts and heal with pitted scars;lesions tend to be in same stage of develop-mentVariola minor variant: constitutional symp-toms, with fewer and smaller skin lesions


Varicella; other viral exanthems, includingcoxsackievirus, parvovirus; infectiousmononucleosis, rubella and rubeola; her-pes simples virus infection; disseminatedherpes zoster infection; impetigo; ery-thema multiforme; rickettsialpox; Kawa-saki disease; rat bite fever; leukemia; con-tact dermatitis

Therapy

Strict respiratory and contact isolation for17 days

�

; vaccination for contacts in earlyincubation period

References

Patt HA, Feigin RD (2002) Diagnosis and man-agement of suspected cases of bioterrorism: a pediatric perspective. Pediatrics 109(4):685–692

Vascular gigantism

�

Vascular malformation

Vascular malformation

Synonym(s)

Vascular gigantism

;

arteriovenous malfor-mation

Definition

Group of disorders characterized by abnor-malities of arteries, veins, capillaries, orlymphatic vessels, often present at birth,producing characteristic clinical, histo-logic, and radiologic changes

References

Fishman SJ, Mulliken JB (1993) Hemangiomas and vascular malformations of infancy and childhood. Pediatric Clinics of North America 40(6):1177–1200


Venous varix 603

V

Vascular spider

�

Spider angioma

Vegetating bromidism

�


Vegetating potassium bromide toxic dermatitis

�


Venectasia

�


Venous clot

�

Thrombophlebitis, superficial

Venous eczema

�

Stasis dermatitis

Venous lake

Synonym(s)

Venous-lake angioma

;

Bean-Walsh angi-oma

;

venous varix

;

senile hemangioma ofthe lips

Definition

Bluish-purple papule secondary to vasculardilatation, occurring usually in elderly peo-ple with excess sun exposure

Pathogenesis

Alteration of vascular and dermal elasticfibers secondary to solar damage, causingvascular dilatation


Well demarcated, blue-purple, soft, com-pressible, smooth papules, distributed onthe sun-exposed surfaces of face and neck,especially on helix or antihelix of ear, pos-terior pinna, or vermilion border of lowerlip


Hemangioma; blue nevus; mucosal melano-sis; melanoma; angiokeratoma circumscrip-tum; traumatic tattoo

Therapy

Cryosurgery; electrosurgery, surgical exci-sion; flashlamp pulse dye laser ablation;intense pulse light ablation

References

Requena L, Sangueza OP (1997) Cutaneous vascu-lar anomalies. Part I. Hamartomas, malforma-tions, and dilation of preexisting vessels. Journal of the American Academy of Dermatol-ogy 37(4):523–549

Venous stasis dermatitis

�

Stasis dermatitis

Venous varix

�

Venous lake


604 Venous-lake angioma

Venous-lake angioma

�

Venous lake

Vermiculate atrophoderma

�


Verruca

�

Wart

Verruca vulgaris

�

Wart

Verrucous carcinoma

Synonym(s)

Ackerman tumor

;

Ackerman’s tumor

;

carcinoma cuniculatum

;

warty cancer

;

epithelioma cuniculatum

Definition

Low grade squamous cell carcinoma char-acterized by slow growth of a verrucousnodule or plaque and rare metastatic spread

Pathogenesis

May be related to human papillomavirus(HPV) infection (particularly on penis,vulva, and periungual region), chemicalcarcinogens, and/or chronic irritation andinflammation, such as that occurring inpatients who chew tobacco or betel nuts oruse snuff


Oral florid papillomatosis variant: white,translucent plaque on erythematous base,located on buccal mucosa, alveolar ridge,upper and lower gingiva, floor of mouth,tongue, tonsil, vermilion border of lip;sometimes develops in previous areas ofleukoplakia, lichen planus, chronic lupuserythematous, cheilitis, or candidiasis;lesions evolve into white, cauliflower-likepapillomas with a pebbly surface, some-times extending and coalescing over largeareas of the oral mucosa; ulceration, fistula-tion, and invasion locally into soft tissuesand boneAnourologic type (Buschke-Loewensteintumor): most commonly on the glans penis,mainly in uncircumcised men; may alsooccur in the bladder and the vaginal, cervi-cal, perianal, and pelvic organs; large, cauli-flower-like nodulePalmoplantar variant (epithelioma cunicu-latum): most commonly involves skin over-lying the first metatarsal head, but also ontoes, heel, medioplantar region, and ampu-tated stumps; exophytic tumors with ulcer-ation and sinuses draining foul-smellingdischarge; pain; bleeding; difficulty walking


Wart; keratoacanthoma; North Americanblastomycosis; leishmaniasis; leprosy;actinomycosis; tuberculosis; mycetoma;granular cell tumor

Therapy

Mohs micrographic surgery�; destructionby electrodesiccation and curettage or liq-uid nitrogen cryotherapy; local radiationtherapy

References Kanik AB, Lee J, Wax F, Bhawan J (1997) Penile

verrucous carcinoma in a 37-year-old circum-cised man. Journal of the American Academy of Dermatology 37(2 Pt 2):329–331

Miller SB, Brandes BA, Mahmarian RR, Durham JR (2001) Verrucous carcinoma of the foot: a re-view and report of two cases. Journal of Foot & Ankle Surgery 40(4):225–231


Vitiligo 605

V

Verrucous dermatitis


Verruga peruana

� Bartonellosis

Vesicular eczema of palms and soles


Vesicular palmoplantar eczema


Vesicular rickettsiosis

� Rickettsialpox

Viking disease

� Dupuytren’s contracture

Vilanova disease


Vincent’s ulcer

� Tropical phagedenic ulcer

Viral keratoses


Vitamin B 3 deficiency

� Pellagra

Vitamin C deficiency syndrome

� Barlow’s disease� Scurvy

Vitiligo

Synonym(s)White spot disease

Vitiligo. Depigmented patch on the upper lip


606 Vogt-Koyanagi-Harada syndrome

DefinitionAcquired progressive leukoderma, charac-terized by depigmented patches

PathogenesisTheories of causation: aberration ofimmune surveillance, melanocyte destruc-tion by neurochemical mediator, melano-cyte destruction by intermediate or meta-bolic product of melanin synthesis, inbornmelanocyte abnormality

Clinical manifestationSharply circumscribed, white macules andpatches, sometimes with perilesionalhyperpigmentation, beginning with fewlesions and expanding over timeLocalized variant: restricted to one area,often in segmental distribution; onset earlyin life, then spreading rapidly withinaffected area; patches persist indefinitelyGeneralized variant: bilaterally symmetri-cal, white macules and patches; sometimesinvolve mucous membranes, particularlythe lip and genitalia; occur in areas ofminor trauma (Koebner phenomenon),such as elbow, knee, dorsal aspect of hands;periorificial location of involvement; depig-mentation of body hair, including scalp,eyebrow, and pubic and axillary hair

Differential diagnosisNevoid hypomelanosis; leprosy; piebald-ism; tinea versicolor; post-inflammatoryhypopigmentation; pityriasis alba; halonevus; scleroderma; lichen sclerosus; tuber-ous sclerosis

TherapyPhotochemotherapy; corticosteroids, topi-cal, superpotent; skin transplants for localareas of depigmentation; widespreadinvolvement: 20 % monobenzylether ofhydroquinone applied twice daily for 3–12months to induce total depigmentation

ReferencesShaffrali F, Gawkrodger D (2000) Management of

vitiligo. Clinical & Experimental Dermatology 25(8):575–579


Synonym(s)Harada syndrome; uveoencephalitis;uveomeningoencephalitic syndrome

DefinitionSyndrome involving various organs con-taining melanocytes, producing uveitis inassociation with cutaneous, neurologic, andauditory abnormalities

PathogenesisMay be a post-viral syndrome, perhaps sec-ondary to Epstein-Barr virus; possibly anautoimmune disorder; susceptibility relatedto presence of HLA-DR4 antigen andDRB*0405 allele

Clinical manifestationProdromal stage: non-specific symptoms,including headache, vertigo, nausea, nuchalrigidity, vomiting, and low-grade feverMeningoencephalitis phase: generalizedmuscle weakness; hemiparesis; hemiplegia;dysarthria; aphasia, and other mental sta-tus changesOphthalmic-auditory phase: decreasedacuity; eye pain and irritation; dysacusis,usually bilateral; tinnitusConvalescent phase: cutaneous signs devel-oping after uveitis begins to subside; polio-isis; vitiligo; halo nevi; alopecia

Differential diagnosisAlezzandrini’s syndrome; piebaldism; vitil-igo; alopecia areata

TherapyHypopigmentation: photochemotherapy;corticosteroids, topical, superpotent; eyeinflammatory changes: prednisone

ReferencesRead RW (2002) Vogt-Koyanagi-Harada disease.

Ophthalmology Clinics of North America 15(3):333–341


Vulvodynia 607

V

Vohwinkel syndrome

Synonym(s)Vohwinkel’s syndrome; keratodermahereditaria mutilans; palmoplantar kerato-derma mutilans

DefinitionDisorder characterized by hyperkeratosis ofthe palms and soles with a honeycombappearance, constrictions of the skinaround the digits, and hyperkeratoticplaques over the dorsal aspects of theextremities

PathogenesisAutosomal dominant trait; phenotype dueto abnormal gap junctions caused by themutation D66H in the gene GJB2 encodingconnexin 26; possibly also insertionalmutation of the loricrin gene

Clinical manifestationHoneycomb-like hyperkeratosis of thepalms and soles; constriction of skinaround digits, causing autoamputation(pseudo-ainhum); starfish-shaped hyperk-eratotic plaques on the dorsum of the handsand feet, elbows, and knees; occasionaldeafness

Differential diagnosisErythropoetic protoporphyria; discoidlupus erythematosus; mal de Meleda; pach-yonychia congenita; palmoplantar kerato-derma of Sybert; Olmsted syndrome; pal-moplantar keratoderma of GamborgNielsen; hereditary bullous acrokeratoticpoikiloderma of Weary-Kindler; Cloustonsyndrome; psoriasis

TherapySurgical release of constriction bands topreserve digits�; acitretin

ReferencesSolis RR, Diven DG, Trizna Z (2001) Vohwinkel's

syndrome in three generations. Journal of the American Academy of Dermatology 44(2 Sup-pl):376–378

Vohwinkel’s syndrome

� Vohwinkel syndrome

Von Frey's syndrome


Von Recklinghausen disease


Von Recklinghausen's disease


Vulvodynia

DefinitionVulvar discomfort, characterized by itch-ing, burning, stinging, or stabbing in thearea around the opening of the vagina

ReferencesMasheb RM, Nash JM, Brondolo E, Kerns RD

(2000) Vulvodynia: an introduction and criti-cal review of a chronic pain condition. Pain 86(1-2):3–10


W

Waardenburg syndrome

Synonym(s)

Klein-Waardenburg syndrome

;

Waarden-burg’s syndrome

Definition

Hereditary disease characterized by deaf-ness in association with pigmentary abnor-malities and other defects of neural crest-derived tissues

Pathogenesis

Autosomal dominant inheritance; unclearcause, but may be related, in part, to devel-opmental defect of neural crest


Type I variant: dystopia canthorum; nasaland other facial abnormalities; strabismusType II variant: normally placed canthi;sensorineural hearing loss; heterochromicirides; white forelock; hypopigmented skinpatchesType III variant: changes of type I variantand the following – musculoskeletal abnor-malities; mental retardation; microcephalyType IV variant: association of changes ofWaardenburg’s syndrome with Hirshs-prung disease


Oculocutaneous albinism; piebaldism; vitil-igo; Woolf syndrome; Fisch syndrome; Roz-lycki syndrome

Therapy


References

Newton VE (2002) Clinical features of the Waardenburg syndromes. Advances in Oto-Rhino-Laryngology 61:201–208

Waardenburg’s syndrome

�

Waardenburg syndrome


Synonym(s)

Waldenström’s macroglobulinemia

;

Wal-denström’s hypergammaglobulinemia

;

Wal-denström hypergammaglobulinemia

Waardenburg syndrome.

Heterochromic irides


610 Waldenström’s hypergammaglobulinemia

Definition

B lymphoma that causes overproduction ofmonoclonal macroglobulin

Pathogenesis

IgM-induced hyperviscosity of blood andneoplastic lymphoplasmacytic cell infiltrat-ration of tissue, leading to many of thesymptoms and signs of the disease


Insidious constitutional signs and symp-toms skinSkin manifestations: purpura; vesicles; andbullae; papules on extremities; chronic urti-caria; Raynaud phenomenon; livedo reticu-laris; acrocyanosisNeurologic findings: mental status change;visual changes; peripheral neuropathyGastrointestinal findings: malabsorption;bleeding; diarrheaPulmonary findings: nodules, masses,parenchymal infiltrates; pleural effusion


Myeloma; other hyperviscosity syndromes;polyarteritis nodosa; Churg-Strauss syn-drome; antiphospholipid antibody syn-drome; serum sickness; septic vasculitis;systemic lupus erythematosus; sarcoidosis

Therapy

Symptomatic hyperviscosity: plasmapher-esis

�

; lymphoma: chemotherapy; splenec-tomy

References

Alexanian R, Weber D (2001) Recent advances in treatment of multiple myeloma and Walden-ström's macroglobulinemia. Biomedicine & Pharmacotherapy 55(9-10):550–552

Waldenström’s hypergammaglobulinemia

�


Waldenström’s macroglobulinemia

�


Warfarin skin necrosis

�

Coumarin necrosis

Wart

Synonym(s)

Verruca

Definition

Virally induced, benign proliferation ofskin and mucosa

Pathogenesis

Caused by human papilloma virus (HPV);various wart subtypes have tendency to besite-specific; viral replication in differenti-ated epithelial cells in upper epidermis


Common variant (verruca vulgaris): hardpapules with a rough, irregular, scaly sur-face, most commonly seen on handsFiliform variant: elongated, slender papuleswith filiform fronds, usually seen on face,around the lips, eyelids, or naresPalmoplantar warts (myrmecia): small,shiny papules, progressing to deep endo-phytic, sharply defined, round papules orplaques with keratotic surface, surroundedby a smooth collar of thickened horn;plantar lesions usually found on weight-bearing areas, such as metatarsal head andheel; hand lesions often are subungual orperiungualFlat wart (plane wart, verruca plana) vari-ant: flat or slightly elevated, flesh-colored,


Water wart 611

W

smooth or slightly hyperkeratotic papules;sometimes become grouped or confluent;may appear in linear distribution as a resultof scratching or trauma (Koebner phenom-enon)Butcher's wart variant: seen in people whohandle raw meat; similar morphology tocommon warts, most commonly on thehandsMosaic variant: plaque of closely groupedwarts, usually seen on palms and solesAnogenital (condyloma accuminata) vari-ant: pink-to-brown, exophytic, cauliflower-like papules or nodules of genitalia, peri-neum, crural folds, and/or anus; discrete,flesh-colored or hyperpigmented papuleson the shaft of the penis; lesions mayextend into the vagina, urethra, cervix, per-irectal epithelium, anus, and rectum


Acquired digital fibrokeratoma; actinickeratosis; squamous cell carcinoma; arseni-cal keratosis; seborrheic keratosis; acro-chordon; lichen planus; molluscum contagi-osum; prurigo nodularis; callus; lichennitidus; acne vulgaris

Therapy

Salicylic acid 5–40 % solution applied dailyfor weeks to months; cantharidin appliedonce every 3–6 weeks; squaric acid applied1–2 times weekly after sensitization; trichlo-roacetic acid 80 % applied once every 4–6weeks; podofilox; imiquimod; bleomycin:0.5–1 unit per ml intralesional injection; liq-uid nitrogen cryotherapy; destruction byelectrodesiccation and curettage; CO

2

laservaporization; hypnotherapy; hyperthermia

References

Allen AL, Siegfried EC (2000) What's new in hu-man papillomavirus infection. Current Opin-ion in Pediatrics 12(4):365–369

Warty cancer

�

Verrucous carcinoma

Warty dyskeratoma

Synonym(s)

Focal acantholytic dyskeratosis

Definition

Solitary, benign, epithelial neoplasm, char-acterized by papule with depressed andcrusted center containing a keratotic plug

Pathogenesis

Localized abnormal keratinization, withunknown stimulus


Flesh-colored to brown papule with centralkeratotic plug, occurring in associationwith the pilosebaceous unit, especially onscalp, face, neck, and axilla; most oftenoccurs in older men


Wart; keratoacanthoma; squamous cell car-cinoma; actinic keratosis; Darier disease;Hailey-Hailey disease; epidermal nevus;Grover’s disease

Therapy

Surgical excision

�

References

Kaddu S, Dong H, Mayer G, Kerl H, Cerroni L (2002) Warty dyskeratoma – “follicular dysk-eratoma”: analysis of clinicopathologic features of a distinctive follicular adnexal neoplasm. Journal of the American Academy of Dermatol-ogy 47(3):423–428c

Wasp sting

�

Hymenoptera sting

Water wart

�



612 Water-rat trapper’s disease

Water-rat trapper’s disease

�

Tularemia

Watson syndrome

Synonym(s)

Watson’s syndrome

;

neurofibromatosis-Noonan syndrome

;

neurofibromatosis withNoonan phenotype

;

pulmonic stenosis

;

caféau lait spots syndrome

Definition

Hereditary condition characterized by clini-cal elements of both Noonan’s syndromeand neurofibromatosis

Pathogenesis

Autosomal dominant trait; may be associ-ated with NF-1 gene mutation


Café-au-lait macules; axillary freckling;Lisch nodules; pulmonary stenosis; lowintelligence; short stature


Neurofibromatosis; Noonan’s syndrome;Turner’s syndrome

Therapy


References

Conway JB, Posner M (1994) Anaesthesia for cae-sarean section in a patient with Watson's syn-drome. Canadian Journal of Anaesthesia 41(11):1113–1116

Watson’s syndrome

�

Watson syndrome


Synonym(s)


;

relapsingfebrile nodular nonsuppurative panniculi-tis

;

nodular nonsuppurative panniculitis

;

Pfeifer-Weber-Christian syndrome

Definition

Spectrum of disorders characterized bynodular panniculitis and additional symp-toms and signs involving multiple organsystems of the body

Pathogenesis

Unknown


Erythematous, edematous, and tender sym-metrical, subcutaneous nodules, usually onthe lower extremities, resolving over a fewweeks, leaving atrophic depressed scar;occasional breakdown of nodules with dis-charge of oily liquid; hepatomegaly;splenomegaly; systemic symptoms: malaise,fever, nausea, vomiting, abdominal pain,weight loss, bone pain, myalgia, andarthralgia


Thrombophlebitis; vasculitis; sarcoidosis;alpha-1-antitrypsin deficiency panniculitis;polyarteritis nodosa; eosinophilic fasciitis;eosinophilic myalgia syndrome; erythemainduratum; erythema nodosum; leukemia;lipodermatosclerosis; lymphoma; pancre-atic panniculitis; poststeroid panniculitis;scleroderma panniculitis; cytophagic histi-ocytic panniculitis; Sweet’s syndrome

Therapy

Prednisone; hydroxychloroquine; azathio-prine; thalidomide; cyclophosphamide;mycophenolate mofetil

References

Enk AH, Knop J (1998) Treatment of relapsing id-iopathic nodular panniculitis (Pfeifer-Weber-Christian disease) with mycophenolate mofetil.


Well’s syndrome 613

W


Wegener granulomatosis

�


Wegener’s disease

�



Synonym(s)

Wegener granulomatosis

;

Wegener’s dis-ease

;

systemic vasculitis

;

systemic necrotiz-ing angiitis

;

necrotizing granulomatousinflammation of the respiratory tract

;

necrotizing glomerulonephritis

Definition

Multisystem disease characterized bynecrotizing granulomatous inflammation ofthe upper and lower respiratory tract, kid-neys, and skin, and by necrotizing vasculi-tis affecting small- and medium-sized ves-sels

Pathogenesis

Probably an autoimmune inflammatoryprocess, antineutrophil cytoplasmic anti-bodies (c-ANCA) directed at neutrophilproteinase 3 (PR-3) may be involved;endothelial cell damage and activation ofneutrophils produce inflammatory media-tors, leading to recruitment of monocytesand T cells and endothelial damage


Non-specific constitutional symptoms andsigns

Skin findings: variable and usually nonspe-cific; palpable purpura; papules; subcutane-ous nodules; ulcerations resembling pyo-derma gangrenosum; petechiae; vesicles;pustules; hemorrhagic bullae; livedo reticu-laris; lower extremities most commonlyaffectedOcular findings: conjunctivitis; scleritis;proptosisEar, nose, and throat findings: sinusitis anddisease in the nasal mucosa, with purulentor sanguinous nasal discharge; otitis media;deformation or destruction of the pinnae ornoseOral findings: mucosal ulcerations; gingivalhyperplasia with petechiaeRenal findings: oliguria; hematuria;glomerulonephritis; chronic renal insuffi-ciencyCardiac and neurologic involvement


Churg-Strauss disease; acute febrile neu-trophilic dermatosis; polyarteritis nodosa;cryoglobulinemic vasculitis; lethal midlinegranuloma; lymphomatoid granulomato-sis; Henoch-Schönlein purpura; pyodermagangrenosum

Therapy

Cyclophosphamide

�

; prednisone

References

Regan MJ, Hellmann DB, Stone JH (2001) Treat-ment of Wegener’s granulomatosis. Rheumatic Diseases Clinics of North America 27(4):863–886

Weil disease

�

Leptospirosis

Well’s syndrome

�



614 Wells syndrome

Wells syndrome

�


Wells’ syndrome

�


Wen

�

Epidermoid cyst

�

Pilar cyst

Werther’s tumor

�


Whirlpool folliculitis

�

Hot tub folliculitis

White folded gingivostomatitis

�

White sponge nevus

White piedra

�

Piedra

White sponge nevus

Synonym(s)

Oral epithelial nevus

;

white folded gingi-vostomatitis

;

hereditary leukokeratosis

;

Cannon’s disease

;

nevus of Cannon

Definition

Developmental mucosal disorder, charac-terized by sponge-like, white plaque onbuccal mucosa and other mucosal sites

Pathogenesis

Autosomal recessive trait; mutation in themucosal keratin K4


Bilateral, white, keratotic plaques, mostcommonly on buccal mucosal surface andsometimes on labial, lingual, and othermucosal sites; thick, white, often corru-gated plaque, sometimes covering much ofbuccal mucosa; occasional less thick lesionswith semitransparent appearance


Hereditary benign intraepithelial dyskera-tosis; Witkop's disease; pachyonychia con-genita; dyskeratosis congenita; leu-koedema; smokeless tobacco keratosis;chronic cheek bite keratosis; leukoplakia


ReferencesMarcushamer M, King DL, McGuff S (1995) White

sponge nevus: case report. Pediatric Dentistry 17(7):458–459

White sponge nevus of Cannon



Winer’s pore 615

W

White spot disease

� Vitiligo

Whitmore disease


Wild hare disease

� Tularemia

Wilson disease

Synonym(s)Hepatolenticular degeneration; Wilson’sdisease

DefinitionInherited disorder of copper metabolism,characterized by cirrhosis and central nerv-ous system degenerative changes

PathogenesisAutosomal recessive trait; gene linked to thelong arm of chromosome 13; defective pro-tein (p-type adenosine triphosphatase)responsible for copper transport; organdysfunction from inadequate biliary cop-per excretion and subsequent copper depo-sition, most notably in liver and centralnervous system

Clinical manifestationSkin changes: hyperpigmentation; bluishdiscoloration over proximal fingernailsGastrointestinal changes: hepatic insuffi-ciency and cirrhosis, with subsequentascites, spider angiomas, palmar erythema,digital clubbing, and jaundice

Ocular findings: copper granules in thestromal layer of the eye (Kayser-Fleischerrings); golden brown, brownish green,bronze color in the limbic area of the eyeCentral nervous system changes: drooling;dysphagia; dystonia; incoordination; diffi-culty with fine motor tasks; masklike facies;gait disturbanceSkeletal abnormalities: highly variable,including osteoporosis, osteomalacia, rick-ets, spontaneous fractures, and polyarthri-tis

Differential diagnosisAutoimmune hepatitis; viral hepatitis; gly-cogen storage disease; multiple sclerosis;Huntington disease; Parkinson disease; leu-kodystrophy; hemochromatosis

TherapyPenicillamine: 250 mg PO 4 times daily�;dietary copper restriction

ReferencesSubramanian I, Vanek ZF, Bronstein JM (2002)

Diagnosis and treatment of Wilson's disease. Current Neurology & Neuroscience Reports 2(4):317–323

Wilson’s disease

� Wilson disease

Winer’s dilated pore

� Dilated pore

Winer’s pore

� Dilated pore


616 Winter erythrokeratolysis

Winter erythrokeratolysis


Winter itch

� Asteatosis� Asteatotic eczema

Wiskott-Aldrich syndrome

Synonym(s)Aldrich syndrome

DefinitionHereditary disorder, characterized byimmunodeficiency, thrombocytopenia,eczema, and recurrent pyogenic infections

PathogenesisX-linked trait; mutations in WASP gene,important transcription factor of lym-phocyte and platelet function; eczemarelated to the abnormal T-cell function andhumoral immune responses

Clinical manifestationEczema with onset in first month of life,indistinguishable from atopic dermatitis;thrombocytopenia and platelet dysfunc-tion at birth, with bloody diarrhea, hematu-ria, epistaxis, and cutaneous petechiae;recurrent bacterial infections beginning ininfancy, with susceptibility to wide varietyof bacterial infections, including septi-cemia, pneumonia, meningitis, pansinusi-tis, conjunctivitis, furunculosis, otitisexterna, and otitis media

Differential diagnosisAtopic dermatitis; Leiner disease; DiGeorgesyndrome; seborrheic dermatitis; Langer-hans cell histiocytosis

TherapyEczema: corticosteroids, topical, midpotency; platelet transfusions as needed;antibiotics for recurrent pyogenic infec-tions; bone marrow transplantation forsevere involvement

ReferencesOchs HD (2001) The Wiskott-Aldrich syndrome.

Clinical Reviews in Allergy & Immunology 20(1):61–86

Wolhynia fever

� Trench fever

Woolsorter's disease


Wooly hair nevus

Synonym(s)None

DefinitionSporadic anomaly of hair growth, charac-terized by coarse, lusterless, and wiry patchof hair

PathogenesisUnknown

Clinical manifestationLocalized area of coarse, wiry hair, usuallylimited to scalp; begins in early childhoodand stable throughout life; sometimes seenwith incontinentia pigmenti

Differential diagnosisMenke’s kinky hair syndrome; uncombablehair syndrome


Wyburn-Mason syndrome 617

W


ReferencesAl Harmozi SA; Somaia FM, Ejeckam GC (1992)

Woolly hair nevus syndrome. Journal of the American Academy of Dermatology 27(2Pt1):259–260

Woringer-Kolopp disease


Wyburn-Mason syndrome

Synonym(s)Bonnet-Dechaume-Blanc syndrome

DefinitionDisease characterized by arteriovenousmalformations in the central nervous sys-

tem and the retina and ipsilateral cutane-ous vascular abnormalities

PathogenesisAlterations in capillary and arteriolar net-works by unknown mechanisms

Clinical manifestationSubtle port wine stain in the region of theaffected eye; intracranial vascular malfor-mations; retinal arterial-venous malforma-tions

Differential diagnosisSturge-Weber syndrome; capillary heman-gioma

TherapyFlash-pumped dye laser ablation of portwine stain

ReferencesPatel U, Gupta SC (1990) Wyburn-Mason syn-

drome. A case report and review of the litera-ture. Neuroradiology 31(6):544–546


X

X-linked chronic granulomatous disease

�


X-linked dominant type

�

Conradi disease

X-linked ichthyosis

Synonym(s)


Definition

Hereditary disorder of keratinization, char-acterized by severe scaling, especially onthe extremities

Pathogenesis

X-linked trait; caused by a steroid sulfatasedeficiency resulting from abnormalities inits coding gene (

STS

); retention hyperkera-tosis from delayed dissolution of desmo-somes in the stratum corneum


Onset at birth or in neonatal period; adher-ent brown scaling in widespread distribu-tion produces dirty-appearing skin; scaling

of scalp, preauricular skin, and posteriorneck; flexures sometimes involved, butpalms and soles usually spared; scalingbecomes more evident and assumes a dirty-yellow or brown color with dark, polygo-nal, firmly adherent scale; tends to fade onhead but more prominent on trunk andextremities, particularly on the extensorsurfaces of the legs; asymptomatic cornealopacities; occasional cryptorchidism


Ichthyosis vulgaris; lamellar ichthyosis;xerosis; atopic dermatitis; hygiene problemwith resultant dirty skin

Therapy

Alpha hydroxy acids; emollients

References

Hernandez-Martin A, Gonzalez-Sarmiento R, De Unamuno P (1999) X-linked ichthyosis: an up-date. British Journal of Dermatology 141(4):617–627

Xanthelasma

�

Xanthoma

Xanthogranuloma

�



620 Xanthoma

Xanthoma

Synonym(s)

Xanthomatosis

Definition

Group of disorders characterized by skinlesions with lipid-laden macrophages

Pathogenesis

Alterations in lipoproteins from geneticmutations yield defective apolipoproteins(primary hyperlipoproteinemia) or fromsystemic disorder, such as diabetes mellitus(secondary hyperlipoproteinemia); distri-bution pattern and morphology of lesionsdepend on specific genetic type or underly-ing disease


Xanthelasma palpebrarum variant: asymp-tomatic, symmetrical, soft, velvety, yellow,flat-topped, polygonal papules on andaround eyelids, most commonly in uppereyelid near the inner canthus; may have noassociated lipid abnormality or may beassociated with hyperlipidemia, where anytype of primary hyperlipoproteinemia canbe present; occasional association with sec-ondary hyperlipoproteinemias, such ascholestasisTuberous xanthoma variant: asympto-matic, firm, red-yellow papules or nodulesusually developing in pressure areas, such

as knees, elbows, or buttocks; may coalesceto form multilobated tumors; associatedwith hypercholesterolemia and increasedlevels of LDL, with familial dysbetalipopro-teinemia and familial hypercholesterolemiaor with secondary hyperlipidemias (e.g.,nephrotic syndrome, hypothyroidism)Tendinous xanthoma variant: slowly enlarg-ing subcutaneous nodules around tendonsor ligaments, often over extensor tendons ofthe hands, the feet, and the Achilles ten-dons; sometimes occurs after trauma; asso-ciated with severe hypercholesterolemiaand elevated LDL levels, particularly in thetype IIa form, or secondary hyperlipi-demias such as cholestasisEruptive xanthoma variant: sudden onset ofcrops of small, pruritic, red-yellow papuleson an erythematous base, most commonlyover buttocks, shoulders, and extensor sur-faces of extremities; may spontaneouslyresolve over weeks; associated with hyper-triglyceridemia, particularly with types I,IV, and V (high concentrations of VLDL andchylomicrons) or with secondary hyperlipi-demias, particularly in diabetesPlane xanthoma variant: flat, yellowishpapules, occurring in any site, and some-times covering large areas of face, neck,thorax, and flexures; when palmar creasesinvolved, type III dysbetalipoproteinemialikely diagnosis; may occur with secondaryhyperlipidemias, especially in cholestasis,with monoclonal gammopathy and hyperli-pidemia, particularly hypertriglyceridemiaXanthoma disseminatum variant: occurs innormolipemic patients; begins in adults asred-yellow papules and nodules with a pre-dilection for flexures; sometimes alsooccurs on mucosa of the upper part of theaerodigestive tract; usually resolves sponta-neouslyVerruciform xanthoma variant: normoli-pemic patients with predominantly oralcavity, solitary, papillomatous yellow nod-ule or plaque


Juvenile xanthogranuloma; amyloidosis;lipoid proteinosis; erythema elevatumdiutinum; sarcoidosis; granuloma annu-

Xanthoma.

Yellow-white papules on the upper eyelids


Xeroderma pigmentosum 621

X

lare; necrobiosis lipoidica; necrobiotic xan-thogranuloma; calcinosis cutis; Langerhanscell histiocytosis; rheumatoid nodules;gouty tophi; mastocytosis; lymphoma

Therapy

Xanthelasma: topical trichloroacetic acid;electrodesiccation; laser therapy; excision;verruciform xanthoma; local excision; con-trol of underlying lipid defect or other ill-ness causing lesions to arise

References

Vermeer BJ, Gevers Leuven J (1991) New aspects of xanthomatosis and hyperlipoproteinemia. Current Problems in Dermatology 20:63–72

Xanthoma disseminatum

�

Xanthoma

Xanthoma multiplex

�


Xanthoma naviforme

�


Xanthoma striatum palmare

�

Xanthoma

Xanthoma, tendinous

�

Xanthoma

Xanthoma, tuberous

�

Xanthoma

Xanthomatosis

�

Xanthoma

Xerac-AC

�

Aluminium chloride

Xeroderma

�

Ichthyosis vulgaris

Xeroderma of Hebra

�



Synonym(s)

Kaposi’s dermatosis

;

xeroderma of Hebra

;

angioma pigmentosum et atrophicum

;

atrophoderma pigmentosum

;

melanosislenticularis progressiva

Definition

Disease characterized by extreme photo-sensitivity, pigmentary changes, prematureskin aging, and development of malignanttumors


622 Xerosis

Pathogenesis

Defect in nucleotide excision repair, lead-ing to deficient repair of DNA damaged byultraviolet radiation; seven XP repair geneswith seven complementation groups; localimmunosuppression may be a factor inincreased skin malignancies


Stage 1: after the age of 6 months, onset ofdiffuse erythema, scaling, and freckle-likeareas of increased pigmentationStage 2: poikiloderma causes an appear-ance similar to chronic radiation dermatitisStage 3: numerous malignancies, includingsquamous cell carcinoma, basal cell carci-noma, malignant melanoma, and fibrosar-comaOcular findings: photophobia; conjunctivi-tis; eyelid solar lentigines; ectropion; sym-blepharon with ulceration; vascular ptery-gia; fibrovascular pannus of the cornea; epi-theliomas of the lidsNeurologic findings: electroencephalo-graphic abnormalities; microcephaly; spas-ticity; hyporeflexia or areflexia; ataxia; cho-rea; motor neuron signs or segmentaldemyelination; sensorineural deafness;supranuclear ophthalmoplegia; mentalretardationDe Sanctis Cacchione syndrome: changes ofxeroderma pigmentosum; neurologicabnormalities; hypogonadism; dwarfism


Basal cell nevus syndrome; porphyria;Bloom syndrome; Cockayne syndrome;progeria; Rothmund-Thomson syndrome;lupus erythematosus; polymorphous lighteruption; LEOPARD syndrome; hydroa vac-ciniforme

Therapy

Absolute protection from sun exposurefrom the time of birth; surgical excision ofskin malignancies

�

; isotretinoin; geneticcounseling for families at risk

�

References

Moriwaki S, Kraemer KH (2001) Xeroderma pig-mentosum – bridging a gap between clinic and laboratory. Photodermatology, Photoimmu-nology & Photomedicine 17(2):47–54

Xerosis

�

Asteatosis

Xerotic eczema

�

Asteatotic eczema


Y

Yaws

Synonym(s)

Pian

;

frambesia tropica

;

bouba

;

parangi

;

paru

Definition

Infectious, nonvenereal, treponemal dis-ease, characterized by involvement of skinand bones

Pathogenesis

Caused by Treponema pallidum, subspeciespertenue, serologically and morphologi-cally indistinguishable from organism caus-ing syphilis


Primary stage: incubation period of 9–90days; primary lesion (mother yaw) at site ofinoculation after a scratch, bite, or abra-sion, most commonly on legs, feet, or but-tocks; nontender, occasionally pruritic, redpapule or nodule that ulcerates; satellitelesions may coalesce to form plaque; lym-phadenopathy; fever; joint pain; motheryaw resolves spontaneously in 2–9 months,leaving atrophic scar with central hypopig-mentationSecondary stage: beginning 6–16 weeksafter primary stage, skin lesions (daughteryaws) resembling mother yaw but smaller;periorificial location; lesions expand, ulcer-ate, and exude a fibrinous fluid that dries

into a crust; red, scaly papules and plaquesthat resemble syphilis over any part of thebody; moist lesions in axillae, groin,mucous membranes; papillomas on plantarsurfaces; macules or hyperkeratotic papuleson palms and soles; skeletal involvement:painful osteoperiostitis; fusiform soft tis-sue swelling of the metatarsals and meta-carpals; may develop relapses after healingup to 5 years following infectionLate stage: occurs after 5–15 years oflatency; progressively enlarging, painless,subcutaneous nodules that ulcerate, withwell-defined edges and indurated base withgranulation tissue and yellowish slough;keratoderma of palms and soles; juxtaartic-ular ulcerated gummatous nodules; skeletallesions consisting of hypertrophic periosti-tis, gummatous periostitis, osteitis, andosteomyelitis


Atopic dermatitis; tuberculosis; leishmania-sis; leprosy; psoriasis; sarcoidosis; scabies;tungiasis; warts; syphilis; keratodermasfrom other causes; insect bite reaction;nutritional deficiency

Therapy

Penicillin G benzathine

�

; erythromycin;doxycycline

References

Walker SL, Hay RJ (2000) Yaws-a review of the last 50 years. International Journal of Dermatology 39(4):258–260


624 Yellow jacket sting

Yellow jacket sting

�

Hymenoptera sting


Z

Zinc deficiency syndrome

�


Zinc depletion syndrome

�


Zinsser-Cole-Engman syndrome

�


Zinsser-Engman-Cole syndrome

�


Ziprkowski-Margolis syndrome

Synonym(s)

Albinism-deafness syndrome

Definition

Hereditary syndrome consisting of congen-ital deafness and partial albinism

Pathogenesis

X-linked trait; possibly related to Waarden-burg syndrome; specific pattern of hearingimpairment in carrier females


Patchy hypopigmentation and hyperpig-mentation; congenital neurosensory deaf-ness; no ocular changes


Piebaldism; oculocutaneous albinism;chemical leukoderma; onchocerciasis;pinta; yaws; Waardenburg syndrome; Alez-zandrini syndrome; leprosy; Vogt-Koyan-agi-Harada syndrome

Therapy


References

Shiloh Y, Litvak G, Ziv Y, Lehner T, Sandkuyl L, Hildesheimer, M, Buchris, V, et al. Genetic mapping of X-linked albinism-deafness syn-drome (ADFN) to Xq26.3–q27.1. American Journal of Human Genetics 47: 20–27

Zonal dermatosis

�

Lichen striatus


626 Zoon balanitis

Zoon balanitis

Synonym(s)

Zoon’s balanitis

;

plasma cell balanitis ofZoon

;

Zoon’s disease

;

Zoon’s plasma cellbalanitis

;

balanitis circumscripta plasma-cellularis

;

plasma cell balanitis

;

plasma cellmucositis

Definition

Benign inflammatory dermatosis of thepenis in uncircumcised men, with histo-logic findings of plasma cells in the dermalinfiltrate

Pathogenesis

Theories of causation: friction; trauma;heat; poor hygiene; chronic infection withMycobacterium smegmatis; reactiveresponse to an unknown exogenous orinfectious agent; immediate hypersensitiv-ity response mediated by immunoglobulinE class antibodies; hypospadias


Solitary, shiny, red-orange-to-violaceousplaque of the glans or prepuce of an uncir-cumcised male


Erythroplasia of Queyrat; candidiasis;lichen sclerosus; lichen planus; syphilis;psoriasis; fixed medication reaction

Therapy

Circumcision

�

References

Mallon E, Hawkins D, Dinneen M, Francics N, Fearfield L, Newson R, Bunker C (2000) Cir-cumcision and genital dermatoses. Archives of Dermatology 136(3):350–354

Zoon’s balanitis

�

Zoon balanitis

Zoon’s disease

�

Zoon balanitis

Zoon’s plasma cell balanitis

�

Zoon balanitis

Zoster

�

Herpes zoster

Zygomycosis

�

Mucormycosis


I

Iatrogenic acrodermatitis enteropathica

�


IBIDS

�

Tay syndrome

Ichthyosiform erythroderma with vacuolation

�


Ichthyosiform nevus

�

CHILD syndrome

Ichthyosis

Synonym(s)

None

Definition

Groups of diseases represented by thick,scaly skin

References

Shwayder T (1999) Ichthyosis in a nutshell. Pedi-atrics in Review 20(1):5–12

Ichthyosis bullosa of Siemens

�


Ichthyosis congenita

�

Ichthyosis fetalis

Ichthyosis congenita larva

�

Lamellar ichthyosis

Ichthyosis fetalis

Synonym(s)


;

harlequin baby

;

ichthyosis congenita

;

keratosis diffusa feta-lis; harlequin fetus

PART9.MIF Page 301 Friday, October 31, 2003 10:36 AM

302 Ichthyosis hystrix

Definition

Severe form of congenital ichthyosis, char-acterized by profound thickening of thekeratin layer in fetal skin, producing ahorny shell of platelike scale and contrac-tion abnormalities of the eyes, ears, mouth,and appendages

Pathogenesis

Probable autosomal recessive trait; abnor-mal lamellar granule structure and func-tion; abnormal conversion of profilaggrinto filaggrin


Condition present at birth; skin severelythickened with large, shiny plates of hyper-keratotic scale; deep fissures separate thescales; severe ectropion, leaving the con-junctiva at risk for desiccation and trauma;pinnae sometimes small and rudimentary,or absent; severe traction on lips causeseclabium and fixed open mouth; nasalhypoplasia and eroded nasal alae; limbsencased in the thick membrane, causingflexion contractures of the arms, legs, anddigits; limb motility poor or absent; hypo-plasia of the fingers, toes, and fingernails;temperature dysregulation; heat intoler-ance; occasional hyperthermia; restrictionof chest-wall expansion sometimes resultsin respiratory distress, hypoventilation, andrespiratory failure; dehydration from excesswater loss


Trichorrhexis invaginata; congenital ichthy-osiform erythroderma; lamellar ichthyosis;Conradi's disease; trichothiodystrophy;Sjogren-Larsson syndrome; X-linked ich-thyosis; lamellar ichthyosis; Netherton'ssyndrome

Therapy

Acitretin

References

Singh S, Bhura M, Maheshwari A, Kumar A, Singh CP, Pandey SS (2001) Successful treatment of harlequin ichthyosis with acitretin. Interna-tional Journal of Dermatology 40(7):472–473

Ichthyosis hystrix

�


Ichthyosis hystrix of Curth-Macklin

�


Ichthyosis, lamellar

�

Lamellar ichthyosis

Ichthyosis linearis circumflexa

�

Netherton syndrome

Ichthyosis nacrée

�

Ichthyosis vulgaris


�

X-linked ichthyosis

Ichthyosis nitida

�

Ichthyosis vulgaris


Id reaction 303

I

Ichthyosis palmaris et plantaris

�


Ichthyosis sebacea

�

Lamellar ichthyosis

Ichthyosis simplex

�

Ichthyosis vulgaris

Ichthyosis vulgaris

Synonym(s)

Common ichthyosis

;

autosomal dominantichthyosis

;

hereditary ichthyosis vulgaris

;

ichthyosis simplex; xeroderma

;

pityriasisvulgaris

;

ichthyosis nacrée

;

ichthyosisnitida

;

fish skin ichthyosis

Definition

Hereditary retention hyperkeratosis charac-terized by large, plate-like, scaly plaques

Pathogenesis

Autosomal dominant trait; altered profilag-grin expression leading to retained scale;chemical abnormality correlated withdecreased numbers of keratohyalin gran-ules


Symmetrical, variable scaling; small, fine,irregular, and polygonal scales, often curl-ing at the edges to give the skin a rough feel;

color ranging from white to dirty gray tobrown; most scaling occurring on extensorsurfaces of extremities, with sharp demar-cation between normal flexural folds andsurrounding affected areas; lower extremi-ties generally more affected than upperextremities; on trunk, scaling often morepronounced on back than abdomen; spar-ing of flexural folds; palmoplantar thicken-ing and hyperlinearity; relative sparing offace; improvement in summer or in warmclimate


X-linked ichthyosis; asteatosis; atopic der-matitis; lamellar ichthyosis; sarcoidosis;dermatophytosis; acquired ichthyosis

Therapy

Alpha hydroxy acids; emollients; keratolyt-ics such as salicylic acid; urea

References

Rabinowitz LG, Esterly NB (1994) Atopic dermati-tis and ichthyosis vulgaris. Pediatrics in Review 15(6):220–226

Ichthyosis, X-linked

�

X-linked ichthyosis

Ichthyotic neutral lipid storage disease

�


Id reaction

Synonym(s)

Autoeczematization

,

autosensitization


304 Idiopathic anetoderma of Schweninger and Buzzi

Definition

Acute, generalized reaction to a variety ofstimuli, including infections and inflamma-tory skin diseases

Pathogenesis

Unknown; theories of causation:(1) abnormal immune recognition of autol-ogous skin antigens; (2) increased stimula-tion of normal T cells by altered skin con-stituents; (3) dissemination of infectiousantigen with a secondary response; and(4) dissemination of cytokines from a pri-mary site


Acute onset of a pruritic, symmetrial, ery-thematous, papular or papulovesiculareruption, usually preceded by acute flare ofunderlying dermatitis or infection, at a sitedistant from the primary infection or der-matitis; vesicles sometimes present on thehands or feet; underlying conditions: der-matophytes, mycobacteria, viruses, bacte-ria, parasites, contact dermatitis, stasis der-matitis, or other eczematous processes


Atopic dermatitis; stasis dermatitis; sebor-rheic dermatitis; contact dermatitis; dys-hidrotic eczema; dermatophytosis; scabies;Gianotti-Crosti syndrome; pityriasislichenoides et varioliformis acuta; drugeruption; folliculitis

Therapy

Prednisone

�

; corticosteroids, topical,medium-potency

References

Gianni C, Betti R, Crosti C (1996) Psoriasiform id reaction in tinea corporis. Mycoses 39(7-8):307–308

Idiopathic anetoderma of Schweninger and Buzzi

�

Anetoderma

Idiopathic atrophoderma of Pasini and Pierini

�



Synonym(s)


;

hypomelanosis guttata ideopathica; leuko-dermia lenticular disseminata

;

leukopathiaguttata et reticularis symmetrica

;

seniledepigmented spots

;

symmetric progressiveleukopathy of extremities

Definition

Acquired, benign leukoderma, most com-monly seen in light-skinned women with ahistory of significant chronic sun exposure

Pathogenesis

Possibly related to sun exposure and itseffect on melanocytes; defect of the epider-mal melanin unit, resulting in hypopigmen-tation


Most commonly seen on the legs of fair-skinned, women, but also occurring on thedorsal aspect of the forearms; multiple,confetti-like, hypopigmented macules


Post-inflammatory hypopigmentation;scars; lichen sclerosus; vitiligo; tinea versi-color; flat warts; pinta

Therapy

Corticosteroids, topical, medium potency;tretinoin; cryosurgery; sun avoidance

References

Falabella R (1988) Idiopathic guttate hypomela-nosis. Dermatologic Clinics 6(2):241–247


Imiquimod 305

I

Idiopathic hypereosinophilic syndrome

�


Idiopathic hypertrophic osteoarthropathy

�


Idiopathic inflammatory myopathy

�

Dermatomyositis

Idiopathic lenticular mucocutaneous pigmentatio

�



�


Imiquimod

Trade name(s)

Aldara

Generic available

No

Drug class

Immunomodulator

Mechanism of action

Induction of cytokines, including tumornecrosis factor-

α

, interferon-

α

, interferon-

γ

, IL-1 and IL-6

Dosage form

5% cream


See table

Common side effects

Cutaneous:

burning sensation, irritant der-matitis, pruritus, local pain, hypopigmenta-tion

Imiquimod. Dermatologic indications and dosage


Basal cell carcinoma Apply 3 times weekly Not indicated


Apply every other day for 16 weeks Not indicated

Genital warts Apply 3 times weekly Not indicated

Keloid, post-excision Apply daily to excision site for 8 weeks

Not indicated


306 Immersion foot


None

Drug interactions

None



References

Dahl M (2002) Imiquimod: a cytokine inducer. Journal of the American Academy of Dermatol-ogy 47(9 suppl):205–208

Immersion foot

Synonym(s)

Trench foot

;

sea boot foot

;

paddy-field foot

;

tropical jungle foot

;

foxhole foot

Definition

Condition produced by prolonged expo-sure of the feet to non-freezing, moist,occlusive microenvironment

Pathogenesis

Hyperhydration causes maceration of thestratum corneum; aggravating factors: tightshoes, foot dependency, immobility, dehy-dration, trauma, history of peripheral vas-cular disease; cold exposure causesincreased blood viscosity, thrombosis,ischemia and cell injury

Clinical manifestationCold water immersion foot: pre-hyperemicstage with cyanotic, absent pulses, and cold,waxy feet; hyperemic stage with painfulfeet, bounding pulses, brawny edema; occurseveral hours after removing footwear;post-hyperemic stage with cold sensitivityand hyperhidrosis that lasts from weeks toyears; warm water immersion foot: severelypainful and/or pruritic, edematous, whitewrinkled feet, with sharp demarcationbetween involved and uninvolved skin

Differential diagnosisChilblains; Raynaud phenomenon; frost-bite; sweaty sock dermatitis; pitted keratol-ysis

TherapyBed rest, leg elevation, and drying of feet�

ReferencesWrenn K (1991) Immersion foot. A problem of the

homeless in the 1990s. Archives of Internal Medicine 151(4):785-788


� Urticarial vasculitis

Impetigo

Synonym(s)Impetigo contagiosa, Fox impetigo,impetigo bullosa, impetigo contagiosabullosa

DefinitionBacterial infection of the superficial layersof the epidermis caused by gram-positivebacterial pathogens

Impetigo. Exudative, eroded plaques with honey-colored crusts on the feet


Incontinentia pigmenti 307

I

PathogenesisBullous variant: gram-positive, coagulase-positive, group II Staphylococcus aureus,most often phage type 71; organisms oftenspread from nasal carriage siteNon-bullous variant: in the United States,group A beta hemolytic streptococcal infec-tion and Staphylococcus aureus occur withequal frequency; in other parts of theworld, group A beta hemolytic streptococ-cal infection is most common cause; organ-ism often transmitted through hand con-tact, entering through abraded or other-wise traumatized skin

Clinical manifestationBullous variant: acute onset of vesicles thatenlarge and quickly rupture, often leavinga peripheral collarette ofk scale; occurs inmilieu of hot and humid environmentswith crowded living conditions and poorhygieneNon-bullous variant: fragile vesicle or pus-tule that readily ruptures and becomes ahoney-yellow, adherent, crusted papule orplaque; located around the nose, mouth,and exposed parts of the body, sparing thepalms and soles; regional, tender lymphad-enopathy

Differential diagnosisHerpes simplex virus infection; varicella;dermatophytosis; pediculosis; thermal orchemical burn; erythema multiforme; fixeddrug reaction; arthropod bite reaction;incontinentia pigmenti; scabies; contactdermatitis; cutaneous candidiasis

TherapyBullous variant: dicloxacillin; cephalexin;mupirocin; bacitracinNon-bullous variant: dicloxacillin;cephalexin; erythromycin; mupirocin; baci-tracin



Impetigo bullosa

� Impetigo

Impetigo contagiosa

� Impetigo

Impetigo contagiosa bullosa

� Impetigo

Incontinentia pigmenti

Synonym(s)Bloch-Sulzberger syndrome, Bloch-Sie-mens syndrome

DefinitionHereditary disorder characterized by neu-rologic, ophthalmologic, dental, and cuta-neous abnormalities

Incontinentia pigmenti. Linear, hyperpigmented, verrucous plaques on the leg


308 Incontinentia pigmenti achromians

PathogenesisX-linked dominant, single gene disorder;mutations in NEMO/IKK-g, which encodesa critical component of the nuclear factor-B (NF-B) signaling pathway; patchy distri-bution of skin lesions resulting from tissuemosaicism due to random X-inactivation

Clinical manifestationCutaneous changes:Stage 1: linear, red papules and vesiclesgrouped on an erythematous base, mainlyon the extremitiesStage 2: linear, verrucous plaques on an ery-thematous baseStage 3: streaks and whorls of brown orslate-gray pigmentation along the lines ofBlaschko, particularly on the trunkStage 4: hypopigmented, atrophic, reticu-lated patches, mostly on the lower extremi-ties; lusterless, thin hair; nail dystrophy,ranging from mild pitting or ridging toseverely thickened, abnormally ridgednails; dental abnormalities• Ocular findings: retinal detachment; pro-liferative retinopathy; fibrovascular retro-lental membrane; cataracts; atrophy of theciliary body• Neurologic findings: seizures; develop-mental delay; mental retardation; ataxia,spasticity; microcephaly; cerebral atrophy;hypoplasia of the corpus callosum; periven-tricular cerebral edema

Differential diagnosisStage 1: bullous impetigo; herpes simplexvirus infection; varicella; epidermolysisbullosa; bullous mastocytosis; epidermo-lytic hyperkeratosis; erythema toxicumStage 2: linear epidermal nevus; lichenstriatus; X-linked dominant chondrodys-plasia punctataStage 3: linear and whorled nevoid hyper-melanosis; dermatopathia pigmentosareticularis; Naegeli-Franceschetti-Jadas-sohn syndromeStage 4: hypomelanosis of Ito; focal dermalhypoplasia syndrome


ReferencesTomaraei SN, Bajwa RP, Dhiman P, Marwaha RK

(1995) Incontinentia pigmenti (Bloch-Sulz-berger syndrome): report of a case and review of the Indian literature. Indian Journal of Pedi-atrics 62(1):118–122

Incontinentia pigmenti achromians

� Hypomelanosis of Ito

Indian tick typhus


Infantile acropustulosis

� Acropustulosis of infancy


Synonym(s)Digital fibrous tumor of childhood; Reyetumor; recurring digital fibroma of child-hood

DefinitionBenign, nodular proliferation of fibrous tis-sue occurring almost exclusively on thedorsal and lateral aspects of the fingers ortoes in infants and small children

PathogenesisUnknown

Clinical manifestationSingle or multiple, firm, erythematous,smooth, dome-shaped papules on the dor-sal-lateral aspect of distal phalanges of the


Infantile/childhood eosinophilic pustulosis of the scalp 309

I

fingers and toes; sparing of the thumbs andgreat toes; occasional spontaneous regres-sion

Differential diagnosisAcquired digital fibrokeratoma; wart;knuckle pad; dermatofibroma; granulomaannulare; angiofibroma; fibrosarcoma; xan-thoma; neurilemmoma; sarcoidosis

TherapySurgery only if impairment or deformity ofthe digits; triamcinolone 3–5 mg per ml int-ralesional

ReferencesKawaguchi M, Mitsuhashi Y, Hozumi Y, Kondo S

(1998) A case of infantile digital fibromatosis with spontaneous regression. Journal of Der-matology 25(8):523–526

Infantile eczema


Infantile eruptive papulous dermatitis




Infantile hemangioma


Infantile lichenoid acrodermatitis


Infantile myofibromatosis


Infantile papular acrodermatitis


Infantile scurvy


Infantile vegetating halogenosis


Infantile/childhood eosinophilic pustulosis of the scalp



310 Infection by achlorophillic algae

Infection by achlorophillic algae

� Protothecosis, cutaneous

Inflammatory angiomatous nodules


Inflammatory linear verrucous epidermal nevus

� Epidermal nevus

Insect bite reaction

� Papular urticaria



Interferon-αααα

Trade name(s)Roferon A; Intron A

Generic availableNo


Mechanism of actionAnti-viral; anti-proliferative; immunoregu-latory

Dosage formPowder for reconstitution for subcutaneousor intramuscular injection


Common side effectsGeneral: flu-like symptomsLaboratory: decreased white blood cellcount, elevated liver enzymes

Serious side effectsBone marrow: suppressionImmunologic: autoimmune thyroiditisNeurologic: spastic hemiplegia, mood dis-orders, seizures; peripheral neuropathyPulmonary: toxic effects

Drug interactionsBone marrow suppressants; vinca alka-loids; zidovudine; aminophylline; inter-leukin-2

Contraindications/precautionsHypersensitivity to drug class or compo-nent; pregnancy; autoimmune hepatitis

ReferencesEdwards L (2001) The interferons. Dermatologic

Clinics 19:139–146

Intertriginous inflammation

� Intertrigo

Intertrigo

Synonym(s)Intertriginous inflammation


Intravascular lymphomatosis 311

I

DefinitionSuperficial inflammation of skin caused bymoisture, bacteria, or fungi in the skin folds

ReferencesGuitart J, Woodley DT (1994) Intertrigo: a practi-

cal approach. Comprehensive Therapy 20(7):402–409

Intestinal amebiasis

� Amebiasis

Intra-oral fistula


Intraepidermal adenocarcinoma


Intravascular endothelioma


Intravascular lymphomatosis


Interferon-αααα. Dermatologic indications and dosage


AIDS-associated Kaposi’s sarcoma

30 million IU/m2 subcutaneously or intramuscularly 3 times weekly

Not applicable

Basal cell carcinoma 500,000 IU subcutaneously 3 times weekly for 3 weeks

Not applicable

Behçet’s disease 2 million IU subcutaneously weekly, escalating to 12 million IU over 2 months

Not indicated

Cutaneous T cell lymphoma

1 million IU intralesional weekly for 4 weeks

Not indicated

Genital warts 250,000 IU intralesional twice weekly for 8 weeks

Not indicated


250,000 IU intralesional twice weekly for 8 weeks

Not applicable

High risk melanoma adjuvant therapy



Infantile hemangioma Not applicable 3 million IU subcutaneously daily for up to 18 months

Keloid, post-excision 1.5 million IU intralesional twice over 4 days

1.5 million IU intralesional twice over 4 days


500,000 IU subcutaneously 3 times weekly for 3 weeks

Not applicable


312 Inverted follicular keratosis


Synonym(s)None

DefinitionBenign proliferation characterized by endo-phytic growth and histologic follicular dif-ferentiation

PathogenesisUnknown

Clinical manifestationSolitary, skin-colored papule or nodulewith a smooth or minimally keratotic sur-face, most commonly on the face of middle-aged patients

Differential diagnosisSeborrheic keratosis; wart; squamous cellcarcinoma; keratoacanthoma; basal cell car-cinoma


ReferencesSoylu L, Akcali C, Aydogan LB, Ozsahinoglu C,

Tuncer I (1993) Inverted follicular keratosis. American Journal of Otolaryngology 14(4):247–248

Iododerma

� Halogenoderma

Iron deposition disease

� Hemochromatosis

Ischemic ulcer

� Decubitus ulcer

Isotretinoin

Trade name(s)Accutane; Amnesteem; Sotret

Generic availableNo

Drug classRetinoid

Mechanism of actionInhibition of sebaceous gland differentia-tion and proliferation; reduction in seba-ceous gland activity; normalization of fol-licular epithelial differentiation

Dosage form10 mg, 20 mg, 40 mg capsule


Common side effectsDermatologic: peeling on hands and feet,cheilitis, skin fragility, alopecia, dry skin,pruritus, paronychiaEyes: dry eyes, with contact lens intoler-ance; dry mucous membranesMusculoskeletal: myalgias, arthralgiasLaboratory: hyperlipidemia

Serious side effectsEye: decreased night visionNeurologic: spinal hyperostosis, pseudotu-mor cerebri, mood disorderGastrointestinal: hepatotoxicity, pancreati-tis


Isotretinoin 313

I

Isotretinoin. Dermatologic indications and dosage


Acne conglobata 0.5 mg/kg PO daily for 1 month followed by 1 mg/kg daily for 4 months


Acne necrotica 0.5 mg/kg PO daily for 1 month followed by 1 mg/kg daily for 4 months


Acne vulgaris 0.5 mg/kg PO daily for 1 month followed by 1 mg/kg daily for 4 months





Chloracne 0.5 mg/kg PO daily for 1 month followed by 1 mg/kg daily for 4 months


Darier disease 0.2–0.3 mg/kg PO daily for 1 month, followed by 0.5–1.0 mg/kg daily indefinitely


Dissecting cellulitis of the scalp






Gram negative folliculitis









Lamellar ichthyosis 0.2–0.3 mg/kg PO daily for 1 month, followed by 0.5–1.0 mg/kg daily indefinitely


Lichen sclerosus 0.5 mg/kg PO daily for 1 month followed by 1 mg/kg daily for 4 months





Muir-Torre syndrome 0.5–1.0 mg per kg PO indefinitely 0.5–1.0 mg per kg PO indefinitely


314 Itching purpura of Loewenthal

Genitourinary: major birth defects; pseu-dotumor cerebri

Drug interactionsTretinoin; benzoyl peroxide; car-bamazepine; tetracyclines

Contraindications/precautionsHypersensitivity to drug class or compo-nent; pregnancy; caution in patients withrenal or hepatic dysfunction, history ofpancreatitis or diabetes mellitus; childrenmay be more sensitive to effects on bones,which may prevent normal bone growthduring puberty

ReferencesHirsch RJ, Shalita AR (2001) Isotretinoin dosing:

past, present, and future trends. Seminars in Cutaneous Medicine & Surgery 20(3):162–165

Itching purpura of Loewenthal


Ito, nevus of


Itraconazole

Trade name(s)Sporanox

Generic availableNo



Dosage form100 mg tablet; 10 mg per ml oral solution

Pityriasis rubra pilaris 0.5 mg/kg PO daily for 1 month followed by 1 mg/kg daily for 4 months





Rosacea 10–20 mg PO daily for 4–6 months Not indicated


10–20 mg PO daily or every other day indefinitely

Not indicated

Steatocystoma mutiplex




1 mg per kg PO daily for 4–6 months 1 mg per kg PO daily for 4–6 months


0.5–1.0 mg per kg PO daily for 4–5 months

Not applicable

Isotretinoin. Dermatologic indications and dosage (Continued)



Itraconazole 315

I


Common side effectsCutaneous: skin eruption, vasculitisGastrointestinal: nausea and vomiting,diarrhea, dyspepsia

Laboratory: elevated liver enzymes, hyper-triglyceridemia

Serious side effectsCutaneous: anaphylaxis, Stevens-Johnsonsyndrome reactionGastrointestinal: hepatotoxicity

Itraconazole. Dermatologic indications and dosage


Aspergillosis 200 mg PO daily until clearing Not indicated

Chromoblastomycosis 200 mg twice daily one week per month for 7 months

Not established



3–5 mg per kg PO once daily for 6–12 months

Majocchi granuloma 200 mg PO daily for 4–6 weeks 5 mg per kg PO once daily for 4–6 weeks



5–7 mg per kg PO daily for a minimum of 6 months

Onychomycosis 200 mg PO twice daily one week per month for 3 months

5 mg per kg once daily for 7 consecutive days each month for 3 months

Oropharyngeal candidiasis

200 mg PO daily for 1–2 weeks 5 mg per kg PO once daily for 1–2 weeks

Protothecosis 200 mg PO daily for 2–6 weeks Not established


100 mg PO daily for 6 months 5–7 mg per kg PO daily or divided into 2 doses for 6 months

Sporotrichosis, disseminated

200 mg PO twice daily indefinitely 5 mg per kg PO daily indefinitely

Sporotrichosis, lymphocutaneous variant

100 mg PO twice daily for 4–8 weeks; if no obvious improvement or if evidence of progressive fungal disease occurs, increase dose in 100 mg increments

100 mg PO daily; contintue for at least 1 week following clinical resolution

Tinea capitis 200 mg PO daily for 1–3 weeks 5 mg per kg PO daily for 2–4 weeks

Tinea corporis 200 mg PO daily for 1–3 weeks 5 mg per kg PO once daily for 1–3 weeks

Tinea cruris 200 mg PO daily for 1–3 weeks 5 mg per kg PO once daily for 1–3 weeks

Tinea faciei 200 mg PO daily for 1–3 weeks 5 mg per kg PO once daily for 1–3 weeks

Tinea pedis 200 mg PO daily for 1–3 weeks 5 mg per kg PO once daily for 1–3 weeks

White piedra 100 mg daily until culture-negative Not established


316 Ivermectin

Drug interactionsAmiodarone; amitriptyline; antacids; bar-biturates; buspirone; carbamazepine;cyclosporine; digoxin; glyburide/met-formin; protease inhibitors; phenytoin;pimozide; quinidine; rifampin; statins; sul-fonylureas; tacrolimus; theophylline; vincaalkaloids; warfarin

Contraindications/precautionsHypersensitivity to drug class or compo-nent; use of the following medications – cis-apride, midazolam, triazolam, pimozide,quinidine, dofetilide, lovastatin, simvasta-tin; history of congestive heart failure; cau-tion in patients with cardiovascular or pul-monary disease or impaired liver or renalfunction

ReferencesMoosavi M, Bagheri B, Scher R (2001) Systemic

antifungal therapy. Dermatologic Clinics 19(1):35–52

Ivermectin

Trade name(s)Stromectol



Mechanism of actionIncreases nerve and muscle cell permeabil-ity of targetpathogens



Common side effectsCutaneous: pruritus, skin eruption, edemaLymph nodes: lymphadenopathyNeurologic: dizziness




Referencesdel Giudice P (2002) Ivermectin in scabies. Cur-

rent Opinion in Infectious Diseases 15(2):123–126

Ivermectin. Dermatologic indications and dosage





Onchocerciasis 150 mcg per kg PO for 1 dose 150 mcg per kg PO for 1 dose

Scabies 200 mcg per kg PO for 1 dose, repeat in 10 days


Strongyloidosis 200 mcg per kg PO for 1 dose, repeat in 10 days



Abrikossof's tumor Abscess Academy rash Acanthamebiasis

Documents

Transcript of Abrikossof's tumor Abscess Academy rash Acanthamebiasis