• See Also
  1. Bullous Disease
  2. Chronic Bullous Disease of Childhood
  • Epidemiology
  1. Onset over age 30 years
  • Pathophysiology
  1. Autoimmune bullous disorder
  2. Variant of Chronic Bullous Disease of Childhood
  • Symptoms
  1. Pruritus
  • 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)
  • Labs
  1. Histology
    1. Subepidermal Blisters
    2. Inflammatory infiltrates at basement membrane zone
  2. Immunofluorescence
    1. IgA deposition along basement membrane
  • Management
  1. First Line: Dapsone or Sulfapyridine
    1. See Dapsone (requires G6PD and monitoring of CBC)
  2. Adjunct: Low dose Prednisone initially
  • Course
  1. Variable course that may last for years
  • References
  1. Bickle (2002) Am Fam Physician 65(9):1861-70 [PubMed]
  2. Cotell (2000) Am J Emerg Med 18(3):288-99 [PubMed]