Henoch Schonlein Mulya Blok Alim FK

Henoch-Schnlein Purpura

IntroductionHSP: Most common vasculitic disease of childhoodSystemic small blood vessels vasculitisNon-thrombocytopenic purpura, arthritis, abdominal pain, gastrointestinal bleeding and nephritis

Clinical features : palpable purpuraabdominal paingastrointestinal tract bleeding arthritis nephritis

Medical record CM hospital 1998-200323 cases with HSP

Case, , 9 years old -2 weeksFeverCoughNauseaVomiting-1 weekPainful of lower extremities NauseaVomitingStomachachePurpuric rash CM hospital D/: Leptospirosis 5 days-1 day Purpuric rash on lower extremities >>>Painful of kneesNauseaAbdominal pain Black stool5th Nov 03 Allergy and Immunology CM hospital

Family historyThe first child of 2 siblingsHistory of pregnancy and delivery : normal Basic immunization : completedFather 40 y.o, teacher school graduated, Rp. 1,000,000,-/monthMother 35 y.o, high school graduated, kindergarten teacher

Physical Examination BW 30 kg (P50-75 NCHS) BL 125 cm (P25-50 NCHS)

Alert, pale (-), dyspneu (-), cyanosis (-)HR : 92 x/min, RR : 24 x/min, T : 37,0 oCBP : 110/70 mmHgEye : normalMouth : normal Heart and lungs : normal

Abdomen : epigastric pain (+) liver and spleen not palpableExtremities : Multiple purpuric rash on lower extremities Joint : painful on knees

Laboratory Hb 12 g/dL, Ht 34.3%, L 28,100/L,T 879,000/L, diff. count -/1/-/82/13/5ESR 50 mm/hrUrinalysis : normal

Diagnosis Henoch-Schnlein Purpura

Laboratory Ureum 24 mg/dL, Creatinin 1 mg/dLIgA 166.1 mg/dL (N: 48-104 mg/dL), C3 154.8 mg/dL (N: 55-120 mg/dL), C4 30.7 mg/dL (N: 20-50 mg/dL)Skin biopsy ~ leucocytoclastic vasculitisEndoscopy : esophagitis

Management Dexamethasone 3 x 5 mg ivMethylprednisolone 3 x 8 mg p oRanitidine 2 x 60 mg p o

Follow up On 5th day : Purpuric rash resolvedAbdominal pain Nausea and vomiting (-)On 8th day :Discharged

Follow up 2 weeks laterSymptoms (-), laboratory abnormalities (-)8 months laterPurpuric rash (+)Any pain (-) Prednisone 5 days

EpidemiologyAge: 2 11 years of ageBoys > girlsIncidence: 14 per 100,000 population

Etiology UnknownUpper respiratory tract infection StreptococcusMycoplasma pneumonia, Helicobacter pylori, Campylobacter jejune, Shigella, hepatitis A, B, C virus, varicella, measles, rubella, CMV,Foods, drugs, chemical toxinCase: (suspected) viral infection

Pathogenesis IgA mediated small vessels vasculitisSerum IgA concentration Immune complexes deposit alternative pathwayInflammatory reaction

Clinical manifestationKidney microscopic hematuriaCentral nervous system seizure, headache, intracranial hemorrhagePulmonary and pleural hemorrhagePancreatitisMyocarditisCholecystitis

DiagnosisAmerican College of Rheumatology (ACR) : palpable purpura age 20 years at onset bowel angina biopsy : granulocytes in the walls of arterioles or venulesCase: 9 years old, palpable purpura, nausea, abdominal pain, joint pain Skin biopsy : leucocytoclastic vasculitis

CaseLeptospirosis : common symptoms acute febrile illness severe headache photophobia

Corticosteroid abdominal pain

Differential diagnosisHypersensitivity vasculitis (HV)Palpable purpuraBowel anginaGastrointestinal bleedingHematuriaAge at onset 20 yearsNo history of medications

LaboratoryRoutine laboratory examination : Complete blood count Erythrocyte sedimentation rate (ESR)Benzidin testUrinalysis Renal function test

Case : leucocytosis, thrombocytosis, ESR, IgA and C3

Skin biopsyLeucocytoclastic vasculitis Case : epidermis basket waves dermis predominantly lymphocytes with nuclear dust ~ leucocytoclastic vasculitis

TreatmentSupportive : HydrationBowel restPain controlNutritional status

Corticosteroid : Abdominal painRenal complication Case :dexamethasone 3 x 5 mg IVmethylprednisolone3 x 8 mg orallyranitidine 2 x 60 mg orally

Prognosis Renal complication (-) overall prognosis is good