Henoch-Schnlein Purpura

Introduction

HSP:
Most common vasculitic disease of

childhood
Systemic small blood vessels vasculitis Non-thrombocytopenic purpura, arthritis,

abdominal pain, gastrointestinal bleeding and nephritis

Medical record CM hospital 1998-2003 23 cases with HSP Symptoms cases

Purpuric rash
Joint pain Abdominal pain

23
14 10

GI tract bleeding Nephritis

3 2

Case, , 9 years old

-2 weeks - Fever - Cough - Nausea - Vomiting

-1 week - Painful of lower extremities - Nausea - Vomiting - Stomachache - Purpuric rash CM hospital D/: Leptospirosis 5 days

-1 day - Purpuric rash on lower extremities >>> - Painful of knees - Nausea - Abdominal pain - Black stool

5th Nov 03 Allergy and Immunology CM hospital

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 oC BP : 110/70 mmHg Eye : normal Mouth : normal Heart and lungs : normal

Abdomen

: epigastric pain (+) liver and spleen not palpable

Extremities : 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/-/82/13/5

ESR 50 mm/hr Urinalysis : normal

Diagnosis
Henoch-Schnlein Purpura

Laboratory

Ureum 24 mg/dL, Creatinin 1 mg/dL IgA 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 vasculitis

Endoscopy : esophagitis

Management

Dexamethasone

3 x 5 mg iv

Methylprednisolone 3 x 8 mg p o

Ranitidine

2 x 60 mg p o

Follow up

On 5th day :
Purpuric rash resolved Abdominal pain Nausea and vomiting (-)

On 8th day :
Discharged

Follow up

2 weeks later
Symptoms (-), laboratory abnormalities (-)

8 months later
Purpuric rash (+) Any pain (-)

Prednisone 5 days

Epidemiology

Age: 2 11 years of age

Boys > girls

Incidence: 14 per 100,000 population

Etiology

Unknown Upper respiratory tract infection Streptococcus Mycoplasma pneumonia, Helicobacter pylori, Campylobacter jejune, Shigella, hepatitis A, B, C virus, varicella, measles, rubella, CMV, Foods, drugs, chemical toxin

Case: (suspected) viral infection

Pathogenesis

IgA mediated small vessels vasculitis

Serum IgA concentration

Immune complexes deposit alternative pathway Inflammatory reaction

Clinical manifestation

Skin lesions palpable purpura

2 10 mm in diameter, pruritus <, pressure dependent distribution

Joint pain in ankle and knees Gastrointestinal tract edema, hemorrhage

Clinical manifestation
Kidney microscopic hematuria Central nervous system seizure, headache, intracranial hemorrhage Pulmonary and pleural hemorrhage Pancreatitis Myocarditis Cholecystitis

Diagnosis

American College of Rheumatology (ACR) : palpable purpura age 20 years at onset bowel angina biopsy : granulocytes in the walls of arterioles or venules

Case: 9 years old, palpable purpura, nausea, abdominal pain, joint pain Skin biopsy : leucocytoclastic vasculitis

Case

Leptospirosis : common symptoms acute febrile illness severe headache photophobia Corticosteroid abdominal pain

Differential diagnosis

Hypersensitivity vasculitis (HV)

Palpable purpura Bowel angina Gastrointestinal bleeding Hematuria

Age at onset 20 years

No history of medications

Laboratory

Routine laboratory examination :

Complete blood count Erythrocyte sedimentation rate (ESR) Benzidin test Urinalysis Renal function test

Case : leucocytosis, thrombocytosis, ESR, IgA and C3

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

Treatment

Supportive :
Hydration Bowel rest Pain control Nutritional status

Corticosteroid :
Abdominal pain Renal complication

Case : dexamethasone 3 x 5 mg IV methylprednisolone 3 x 8 mg orally ranitidine 2 x 60 mg orally

Prognosis
Renal complication (-) overall
prognosis is good