Henoch-Schönlein syndrome

  • Acute inflammatory reaction of unknown cause occurring in small blood vessels

Typical Patient

  • Child

Symptoms / Signs

  • purpura on extensor surfaces of limbs, buttocks

  • oedema in face and extremities

  • periarticular tissues of wrists, knees, ankles  swollen / painful

  • gut involvement

    • abdominal pain

    • haematemesis

    • melaena

    • intussusception

  • renal involvement

    • haematuria

    • proteinuria

    • glomerulonephritis → renal failure → hypertensive encephalopathy

Variants

Pathology

  • vasculitis

Investigations

  • FBC

    • platelet count in normal range (distinguishes from other purpura)

  • urinalysis

    • proteinuria

    • haematuria

Treatments

  • analgesia

  • steroids if very extensive lesions

Prognosis

  • excellent

  • usually settles in 5-10 days

  • relapses up to one year

Complications

Henoch-Schönlein purpura

  • Systemic vasculitis
  • seen in children,, rare in adults
  • male:female=2:1
  •  
  • deposition of IgA in arterioles, venules, capillaries
  • occurs after upper respiratory tract infections
  • manifestations include
    • purpura
    • transient, non-migratory polyarthritis
    • abdominal pain
    • 50% have glomerulonephritis (focal segmental proliferative) leading to haematuria and proteinuria
  • Prognosis excellent
 

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