small vessel vasculitis

Etiology

• primary small vessel vasculitis (15-20%)
  • Wegener's granulomatosis (granulomatosis with polyangiitis)
  • Churg-Strauss syndrome (eosinophilic granulomatosis with polyangiitis)
  • microscopic polyangiitis
  • Henoch-Schonlein purpura (IgA vasculitis)
  • leukocytoclastic vasculitis
  • cryoglobulinemia
  • Behcet's syndrome
  • Cogan's syndrome
• connective tissue disease
  • systemic lupus erythematosis
  • rheumatoid arthritis
  • Sjogren's syndrome
• inflammatory bowel disease
• infections (15-20%)
  • group A beta-hemolytic streptococci
  • hepatitis B virus
  • hepatitis C virus (cryoglobulinemia)
  • septic vasculitis
• pharmaceutical agents (10-15%)
  • sulfonamides
  • penicillins
  • allopurinol
  • phenytoin
  • thiazides
  • fluoroquinolones
• malignancy (< 5%)
  • paraproteinemia
  • lymphoproliferative disease
• idiopathic (50%)

Clinical manifestations

• palpable purpura

Laboratory

• full thickness skin biopsy

Differential diagnosis

• primary small vessel vasculitis
  • IgA vasculitis
    • serum complement (C3,C4) normal, ANCA negative, hematuria, no proteinuria, leukocytosis with normal differential, abdominal pain, GI bleed, arthritis
  • cryglobulinemia
    • serum complement low, ANCA negative, cutaneous symptoms 100%, palpable purpura, Raunaud's phenomenon 50%
  • ANCA-associated vasculitis
    • granulomatosis with polyangiitis (Wegener's granulomatosis)
    • eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome)
      • asthma, sinusitis, eosinophilia, ANCA positive in 50%
    • microscopic polyangiitis
      • pANCA, arthralia, myalgia, neuropathy, glomerulonephritis, hemoptysis from pulmonary vasculitis
• polyarteris nodosa
  • skin nodules, skin ulcers (deeper than palpable purpura), myalgia, arthralgia, fever, weight loss, intestinal ischemia, testicular pain

Management

• identify underlying etiology
• remove offending agents
• sparing use of glucocorticoids
• cutaneous small vessel vasculitis (palpable purpura) can be managed with
  • rest & elevation of affected region
  • topical glucocorticoids
  • NSAIDs
  • antihistamines
• see specific etiology

More general terms

• vasculitis

More specific terms

• acute leukocytoclastic vasculitis (Henoch-Schonlein purpura; IgA vasculitis)
• allergic angiitis & granulomatosis of Churg-Strauss; eosinophilic granulomatosis with polyangiitis (EGPA)
• Behcet syndrome
• Cogan's syndrome; diffuse interstitial keratitis
• cryoglobulinemia (cryoglobulinemic vasculitis)
• cutaneous lupus erythematosus
• granulomatosis with polyangiitis; Wegener's granulomatosis
• leukocytoclastic vasculitis; hypersensitivity vasculitis; trisymptome
• microscopic polyangiitis

Additional terms

• large vessel vasculitis (large vessel arteritis)
• medium vessel vasculitis