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