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 manifestation Skin lesions palpable purpura 2 10 mm in diameter, pruritus

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