Feedback Haematology

Henoch-Schönlein Purpura

  • Small vessel vasculitis caused by immune complex (IC) deposition following an infectious trigger
    • MC after infection caused by group A strep
  • Primarily affects the skin and mucous membranes
  • Leukocytoclastic vasculitis, increase incidence in winter

Epidemiology

  • MC in children
  • MC in boys

Pathophysiology

  • Small-vessel vasculitis in which ICs of IgA and C3 are deposited on arterioles, venules and capillaries
    • Type III HSR
  • Involves skin, connective tissue, joints, scrotum, GIT and kidneys
  • Association with vaccination for typhoid, cholera, measles, hep B

Clinical features

  • Classic triad – purpura, arthritis and abdominal pain
    • And GI hemorrhage
  • Purpura is MC on legs and buttocks
  • Abdominal pain – colicky
  • Arthritis – MC on ankles, knees, elbows
  • Kidney involved in 40% of cases
    • Hematuria, proteinuria

Diagnosis

  • Mainly based on combination of symptoms
  • History of infection – group A β-hemolytic strep; HBV; HSV; Measles; H.pylori
  • Blood (non-specific)
    • ↑creatinine and urea – kidney involvement
    • ↑CRP + ESR
  • Immunofluorescence – demonstrates IgA + C3

Treatment

  • Mostly self limiting
  • Analgesia for abdominal and joint pain
  • Corticosteroids – moderate to severe
Feedback