Henoch-Schonlein Purpura

- a.k.a anaphylactoid purpura
- small vessel vasculitis characterised by palpable purpura (usually over buttocks and lower extremities), arthralgia, gastrointestinal signs and symptoms and glomerulonephritis
- presumptive pathogenic mechanism for HSP is immune complex deposition (usually IgA)
- palpable purpura is seen in virtually all paediatric patients
- gastrointestinal involvement is characterised by colicky abdominal pain associated with nausea, vomiting, diarrhea or constipation frequently accompanied by passage of blood and mucus per rectum, bowel intussusception may occur
- diagnosis of HSP is based on clinical signs and symptoms
- skin biopsy : leukocytoclastic vasculitis
- excellent prognosis, 1-5% of children progress to ESRF
- treatment: prednisolone (1mg/kg) has been shown to be useful in decreasing tissue edema, arthralgia and abdominal discomfort
- patients with rapidly progressive glomerulonephritis have been anecdotally reported to be benefit from intensive plasma exchange combined with cytotoxic drugs