II. Epidemiology

  1. Onset over age 30 years

III. Pathophysiology

  1. Autoimmune bullous disorder
  2. Variant of Chronic Bullous Disease of Childhood

IV. Symptoms

V. Signs

  1. Annular Lesions
    1. Papules (may be excoriated and crusted)
    2. Vesicles
    3. Bullae
  2. Distribution
    1. Symmetric involvement on extensor surfaces
    2. Elbows, knees, and buttocks affected
    3. Mucusa involvement may occur (Conjunctiva, oral)

VI. Labs

  1. Histology
    1. Subepidermal Blisters
    2. Inflammatory infiltrates at basement membrane zone
  2. Immunofluorescence
    1. IgA deposition along basement membrane

VII. Management

  1. First Line: Dapsone or Sulfapyridine
    1. See Dapsone (requires G6PD and monitoring of CBC)
  2. Adjunct: Low dose Prednisone initially

VIII. Course

  1. Variable course that may last for years

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