Anaphylactoid purpura- Vasculitis of small vessels

Henoch Schonlein Purpura

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HSP

Typically follows an attack of URI More frequent in children (2-8 yrs) Most frequently in winter months Males twice as frequently as females Incidence 9/100,000 population

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Pathogenesis

IgA mediated vasculitis of small vessels

Deposition of IgA & C3 in small vessels of skin and renal glomeruli

ASO titers increase - ? Group A streptococcus

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Clinical Acute Insidious

Rash – palpable purpura Red – purple – rusty brown – fade Lesions in crops – last from 3-10 days Intervals vary from 3-4 months Local angioedema in dependent or

distensible areas Arthritis in 2/3 knees, ankles Serous effusions – resolve without residual

damage

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Clinical Edema and damage to vasculature of GI

tract leads to pain abdomen – often colicky

Occult heme – positive stools, diarrhoea with or without visible blood or hematemesis

Intessusception with current jelly stools may occur (ileo-ileal)

Renal involvement in 25-50% HSM, lymphadenopathy

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Complications

Rarely CNS involvement Seizures, peresis. Coma

Rheumatoid like nodules Cardiac and eye involvement Pancreatitis Pulmonary or intramuscular

hemorrhages

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Diagnosis

Palpable purpura Crops in dependant areas

DD: Kawasaki disease Polyarteritis nodosa Meningococcemia Systemic JRA Factor V Leiden / Protein C deficiency

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Labs

Normal

Biopsy : confirmatory [cutaneous vasculitis]

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Treatment

Symptomatic Hydration Pain control Avoidance of competitive activities GI symptoms ; IV Corticosteroids :

1-2 mg/kg/hr

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Complications

Renal involvement : Nephrotic syndrome

Bowel perforation Testicular torsion

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Prognosis

Self limiting < 1% develop renal involvement and

, 0.1% develop serious renal disease

Rarely death in acute stage due to bowel infarction

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