D69.0
DESCRIPTION
Henoch Schönlein Purpura (HSP) is an acute leucocytoclastic vasculitis of small blood vessels usually involving skin, gastrointestinal tract, joints and the kidney. Aetiology is unknown.
Complications include:
- acute severe abdominal pain, bowel infarction;
- nephritis with renal impairment or nephrotic syndrome;
DIAGNOSTIC CRITERIA
Clinical
Syndrome consisting of:
- Non-thrombocytopenic palpable purpuric skin rash with a very typical distribution on lower extremities and buttocks. The rash occurs in 100% of cases, but not necessarily present at time of initial presentation. Trunk and upper extremities may be involved. Angio-oedema of scalp, eyelids, lips and ears.
- Arthralgia/arthritis (60–70%): mostly of large joints, i.e. knees and ankles.
- Abdominal pain with colic (60-70%): may develop gastro-intestinal bleeding or intussusception or infarction.
- Renal involvement (25–50%) manifesting with haematuria or proteinuria.
Investigations
- No diagnostic test.
- FBC is usually normal but necessary to rule out other conditions with thrombocytopaenic purpura.
- Coagulation studies are normal.
- Urine test strip to evaluate renal involvement. Serum urea, creatinine, electrolytes and albumin with renal involvement.
- Check stools for occult or frank bleeding.
GENERAL AND SUPPORTIVE MEASURES
- Short period of immobilisation during acute arthritis.
- Soft diet for acute gastrointestinal involvement.
- Clinical review with blood pressure monitoring and urine test strip weekly for first 2 months, then monthly for the next year.
MEDICINE TREATMENT
For arthritis, oedema, fever, malaise:
- Ibuprofen, oral, 10 mg/kg/dose 6 hourly.
- Reduce dose interval to 8 hourly after pain is managed.
For complicated HSP (severe extrarenal symptoms or renal disease):
- Prednisone, oral, 1–2 mg/kg/dose once daily for 10 days in the morning.
- Reduce dose gradually over 2 weeks.
REFERRAL
HSP with complications, i.e. in patients with:
- Persistent proteinuria, persistent macroscopic haematuria or progressive nephritic syndrome (renal biopsy indicated).
- Persistent abdominal pain.