Blister

Epidermolysis Bullosa Acquisita

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Epidermolysis Bullosa Acquisita, Epidermolysis Bullosa

  • See Also
  1. Bullous Disease
  2. Pemphigoid
  • Epidemiology
  1. Mean age of onset: 50 years
  • Pathophysiology
  1. Autoimmune Subepidermal Blistering condition
  • Signs
  1. Trauma prone areas more commonly affected
  2. Tense Blisters and erosions over extensor surfaces
    1. Knuckles
    2. Dorsal hands
    3. Elbows
    4. Knees
    5. Ankles
  3. Mucosal involvement common
    1. Oral, nasal, and esophageal mucosa
    2. Conjunctival mucosa
  • Labs
  1. Histology
    1. Subepidermal Blister with inflammatory infiltrate
  2. Immunofluorescence
    1. IgG deposits at dermal-epidermal junction
  • Complications
  1. Scarring skin lesions (with associated milia)
  • Differential Diagnosis
  1. Porphyria cutanea tarda
  2. Hereditary Epidermolysis Bullosa (non-immune mediated)
  3. Bullous Pemphigoid (non-scarring)
  • Course
  1. Chronic waxing and waning course
  • References
  1. Cotell (2000) Am J Emerg Med 18(3):288-99 [PubMed]