PURUPURA HENOCH SCHONLEIN TIPE BULA HEMORAGIK

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LOGO PURPURA HENOCH SCHONLEIN WITH HEMORRHAGIC BULLOUS SKIN LESION Medina Athiah

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PURUPURA HENOCH SCHONLEIN TIPE BULA HEMORAGIK

Transcript of PURUPURA HENOCH SCHONLEIN TIPE BULA HEMORAGIK

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PURPURA HENOCH SCHONLEIN WITH HEMORRHAGIC BULLOUS SKIN LESIONMedina Athiah

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Correction

Halaman 15 Jawaban konsultasi ke Subdivisi Nefrologi: Kesan: Nefritis PHS seharusnya PHS dengan proteinuria.

Halaman 16, 17, 19, 20 Masalah no. 2 Nefritis PHS seharusnya proteinuria

Halaman 18 proteinuria (-) nefritis PHS perbaikan seharusnya proteinuria (-) proteinuria selesai

Halaman 18 feses rutin darah samar (+) seharusnya darah samar (-)

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Introduction

Henoch-Schonlein Purpura (HSP) or anaphylactoid purpura, is a vasculitis of the small blood vessels that

are often found in children.

PHS incidence ranged from 13.5 to 18 per 100,000 children.

The purpose of this case report presents a case about PHS with the form of hemorrhagic bullae which is quite

uncommon manifestations in children.

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Identification

I/♂8 y.o 11 m.o.

Admitted Nov 30TH 2013

Mr. A31 y.o.

Junior High School

Ojek Driver

Mrs. S29 y.o

Junior High School

House wife

Patient MotherFather

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CC Abdominal pain

Anamnesis

Rash on legsAC

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Anamnesis

44 days b.a days b.a

22 day b.a day b.a

High feverNauseaNo appetiteBrought to the clinic Given 2 kinds of drugs (paracetamol and antibiotics)

Rash on the legs and buttocksJoint pain, especially in the knee and ankle and Could not walkFever.

Present Illness History

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Anamnesis

3 days b.a3 days b.aMore rash on the legs Abdominal pain Fever Nausea Vomiting, 2 times/day, the contents of what is eaten, @ 1/4 glass of water Joint pain at the knee and ankleBrought to the clinic 24 hours given the 4 kinds of drugs (paracetamol, antibiotics, stomachache drug, and anti-allergy drugNo improvement ER of Moh Hoesin hospital

Present Illness History

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Was treated at the Allergy Immunology, Moh Hoesin Hospital with a diagnosis of PHS in 2010

Anamnesis

Past I.H

•No history of same illnessFH

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Anamnesis

Normal

Complete according age and oncourse

Good in quality and quantity

History of Pregnancy and labor

History of immunization

History of nutrition

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Anamnesis

Normal

Middle low income

Good

DevelopmentalDevelopmental

Socio-economic Socio-economic

EnvironmentEnvironment

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Physical Examination

Alert

Pulse 88 b/m (v&p equal)

Temperature 36,8o

Resp. Rate 22 t/m (regular)

Good Nutritional Status

General Findings

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Physical Examination

Head: Normocephal, no anemia, no icteric, pupil: round, isocor, positive reflex, no nasal flare.Thoracic: symmetrical, no retraction.Cor: normal heart sound, no murmur.Pulmo: normal vesicular, no ronchi, no wheezing.

Abdominal: flat, tender, unpalpable liver, unpalpable spleen, epigastric tenderness, tymphani, normal bowel sound.Extremities: warm akral, CRT <2 seconds, multiple purpura, diameter of 1-2 cm scattered on the left and right cruris and dorsum pedis

Spesific Findings Spesific Findings

Normal Neurologic status

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Initial Analysis

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Diagnosis

Henoch-SchonleinPurpura

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Initial Problems

Purpura Henoch-Schonlein-Methyl prednisolone tablet 4 mg tablet 3x2 tablet-Diet 1800 kcal and 50 g protein per day-Monitoring for blood pressure, abdominal pain, bloody stool, rash

Problem(P)

P 1

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Progress Note (1-12-2013)

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Progress Note (3-12-2013)

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Progress Note (4-12-2013)

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Progress Note (5-12-2013)

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Progress Note (6-12-2013)

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Progress Note (7-12-2013)

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Progress Note (11-12-2013)

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Progress Note (12-12-2013)

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Literature Review

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History

Henoch-Schonlein Purpura (PHS) (1801) vasculitis of small blood vessels, was first described by William and Heberden.

Lucas Schonlein (1832) describe abnormalities in the form of purpura and arthralgia.

Eduard Henoch (1874) find a relationship with symptoms of gastrointestinal and genitourinary systems.

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Definition

PHS (anaphylactoid purpura / purpura nontrombositopenik) clinical syndrome caused by systemic small blood vessels vasculitis characterized by specific skin lesions such as purpura nontrombositopenik, arthritis or arthralgia, abdominal pain or gastrointestinal bleeding, and occasionally nephritis or hematuria

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Epidemiology

PHS incidence from 13.5 to 18 per 100,000 children.

PHS occurs at the age of 6 months to adults, 50% of cases occur in children less than 5 years, 75% at age below 10 years

Male: Female = 1.5-2: 1

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Etiology Upper respiratory tract infection (60-75%) Bacteria and viruses such as Streptococcus grup A,

Yersinia sp, Legionela sp, Mycoplasma, Epstein-Barr, Varicella, Parvovirus B19, Adenovirus dan Campylobacter enteritis.

Post-vaccination, such as typhoid and paratyphoid A and B, Measles, Varicella, Rubella, and Hepatitis B.

Drugs, such as Penicillin, Ampicillin, Quinin, etc/ Foods, weather and insect bites. Genetic factors. Malignancies, leukemia and lymphoma.

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Pathophysiology

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Pathophysiology

Pathophysiology of hemorrhagic bullous at PHS remains unclear.

Studies Kobayashi et al. increase in matrix metalloproteinase-9 (MMP-9, gelatinase B) in liquid bullaewhich is examined by zymography

MMP-9 (secreted by PMN in dermoepidermal junction) migrate from intensive vasculitis lesions and form bullae by degrading basal membrane components such as collagen type VII

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Histopathology

Typical picture of a vasculitis leucocytoclastic vasculitis with or without fibrinoid necrosis, until perivascular lymphocytes infiltrates with extravasation of erythrocytes

IgA deposits in the walls of blood vessels specific and sensitive markers of PHS

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Figure 2: A. H & E staining of skin biopsy showed leukocytoclastic vasculitis with neutrophil infiltration B. Direct Imunofloresensi of

skin biopsies showed IgA deposition in blood vessels

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Clinical Manifestations

Skin: palpable purpura, found on the skin buttocks and lower extremities, but can also be on the upper extremities, face and body.

Gastrointestinal tract: abdominal pain, vomiting, colicky abdominal bleeding, gastrointestinal bleeding, intestinal perforation and intussusception ileoileal or ileokolonal

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Clinical Manifestations

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Clinical Manifestations

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Clinical Manifestations

Uncommon form of the lesions in HSP are vesicles containing blood that can develop into bullae containing blood (hemorrhagic)

Only occurs <2% of cases of hemorrhagic bullae in children

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Clinical Manifestations

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Clinical Manifestations

Joints: can not walk, arthralgia, joint swelling, redness and arthritis.

Kidney: hematuria, proteinuria, hypertension and renal failure.

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Supportive Findings

There are no specific laboratory tests for HSP

Routine bloodUrinalysisRenal functionElectroliteIgA level in serumSkin and renal biopsy

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Diagnostic

Based on specific clinical symptoms, laboratory tests, and histopathological.

There are two classification systems for HSP American College of Rheumatology (ACR) and the Chapel Hill Consensus Group

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Henoch-Schonlein Purpura from ACR

Criteria Definitions

Purpura non trombositopenia (Palpable

purpura)

Hemorrhagic skin lesions that can be

touched, there is elevation of the skin, and

not associated with thrombocytopenia

Onset of age ≤ 20 y.o Onset of age ≤ 20 y.o

Abdominal symptomps (Bowel angina) Diffuse abdominal pain, become heavy

after a meal, or a diagnosis of bowel

ischemia, usually including bloody stool

Granulocytes in vessel walls in biopsy Showed histologic changes of granulocytes

in the walls of arterioles or venules

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Management

Supportive and symptomatic, including maintenance of hydration, nutrition, electrolyte balance and relieve pain with analgesics.

Self-limiting disease within a few days-weeks.

Corticosteroids for severe symptoms, arthritis, severe abdominal pain, gastrointestinal bleeding, edema and persistent nephrotic syndrome.

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Management

Management for hemorrhagic bullae on HSP can not be explained with certainty until today.

In general HSP with hemorrhagic bullae still be managed as a case of PHS in general.

The difference is its symptomatic treatment compress with 0.9% NaCl three times a day, topical antibiotics such as gentamicin or fusidic acid ointment

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Case Analysis

Case

• Age: 8 years 11 months• Boy

Literature

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• PHS occurs at the age of 6 months to adults, 50% of cases occur in children less than 5 years, 75% at age below 10 years

• Male: Female = 1.5-2: 1

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Case Analysis

Case

• Palpable skin rash• Abdominal pain• Joint pain• Bloody stools

Literature

• Palpable purpura non trombocytopenia

• Bowel Angina (Diffuse abdominal pain, become heavy after a meal, or a diagnosis of bowel ischemia, usually including bloody stool)

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Case Analysis

Case

• Lab findings:• No trombocytopenia• Leucocytosis• Prolonged of ESR• Proteinuria• Occult blood (+)

Literature

• Lab findings:• No trombocytopenia• Leucocytosis• Prolonged of ESR and

CRP• Proteinuria• Hematuria• Decreased of renal

function in severe complications of renal.

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Case Analysis

Case

• Bed rest• Metil prednisolon 1-2

mg/kgBW/day full dose within 2 weeks

• Gentamicin cream• Monitoring of blood

pressure, urinalysis, and renal function

trum

Literature

• Bed rest• NSAID for arthritis and fever• Kortikosteroid for severe

symptoms• Severe Complications in

renal corticosteroid and immunosuppresants

• For bullae hemorrhagic lesions: compress with normal saline, topical antibiotic

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