leukocytoclastic vasculitis; hypersensitivity vasculitis; trisymptome; immune complex-mediated vasculitis

Etiology

• autoimmune disease
  • systemic lupus erythematosus
• recent viral infection
  • hepatitis C, cryoglobulinemia
• malignancy - lymphoproliferative disorders
• drug adverse effect
• systemic vasculitis
• idiopathic (40%)

Clinical manifestations

• palpable purpura in small bunches over the lower extremities & buttocks
• cutaneous vesicles, pustules, maculopapular lesions, urticaria
• occurs within 2 weeks of exposure to offending agent
  • 50% of cases, the offending agent is unknown
• generally resolves when the offending agent is removed

Laboratory

• skin biopsy
  • neutrophilic infiltrate of small vessels, leukocytoclasia, & fibrinoid necrosis
  • direct immunofluorescence reveals only scant deposits of C3, IgM, IgA, & IgG

Differential diagnosis

• exanthematous drug eruption (morbilliform exanthem)
• immune thrombocytopenic purpura
  • petechiae/purpura of thrombocytopenia are not palpable
• IgA vasculitis:
  • dominant IgA pattern of immunoglobulin deposition vessels
  • systemic features (renal. GI, joint)

Management

• generally self-limited
  • withdrawal of offending agent
• glucocorticoids
• see acute leukocytoclastic vasculitis (Henoch-Schonlein purpura) or specific etiology

More general terms

• vasculitis
• type 3 hypersensitivity; immune complex hypersensitivity

More specific terms

• acute leukocytoclastic vasculitis (Henoch-Schonlein purpura; IgA vasculitis)
• chronic leukocytoclastic vasculitis (Gougerot-Ruiter type)
• cutaneous leukocytoclastic vasculitis
• urticarial vasculitis

Additional terms

• cryoglobulinemia (cryoglobulinemic vasculitis)
• leukocytoclasis
• serum sickness; serum sickness-like reaction