Henoch-Schönlein Purpura (HSP)

This is an idiopathic purpuric ± urticarial rash over buttocks, extensor knees, and ankles associated with:

  • fever and malaise
  • transient arthritis, arthralgia (2/3rds)
  • colicky abdominal pain (50%) ± intussusception
  • gastrointestinal tract bleeding (GITB) → melaena
  • haematuria (renal involvement) (25-50%) → nephrotic syndrome (CRF 2-4%)

75% of cases occur in children under the age of 10 years. Up to 25% of cases, however, may present without purpura. HSP is thought to be an allergic IgA-mediated vasculitis in response to bacteria or drugs.

Management

Most cases are self-limiting and require only symptomatic management but close follow-up is needed to identify significant but often asymptomatic renal involvement requiring intervention.

  • bed rest
  • analgesia
  • regular urinalysis

Causes of Purpura

petechiae

  • vascular defect
    • anaphylactoid purpura
    • infective states
    • vitamin C deficiency
    • hereditary haemorrhagic telangiectasia, polyarteritis nodosa, uraemia, Cushing’s syndrome, Ehlers-Danlos syndrome
  • platelet abnormality
    • idiopathic thrombocytopenic purpura (ITP), secondary thrombocytopenia
    • leukaemia
    • other infiltrates
    • Gram negative sepsis
    • aplastic anaemia

ecchymoses

  • abnormal coagulation
    • haemophilia A & B
    • von-Willebrand disease
    • Christmas disease

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