Epidermolysis Bullosa Acquisita

Aka: Epidermolysis Bullosa Acquisita, Epidermolysis Bullosa

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II. Epidemiology

  1. Mean age of onset: 50 years

III. Pathophysiology

  1. Autoimmune Subepidermal Blistering condition

IV. 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

V. Labs

  1. Histology
    1. Subepidermal Blister with inflammatory infiltrate
  2. Immunofluorescence
    1. IgG deposits at dermal-epidermal junction

VI. Complications

  1. Scarring skin lesions (with associated milia)

VII. Differential Diagnosis

  1. Porphyria cutanea tarda
  2. Hereditary Epidermolysis Bullosa (non-immune mediated)
  3. Bullous Pemphigoid (non-scarring)

VIII. Course

  1. Chronic waxing and waning course

IX. References

  1. Cotell (2000) Am J Emerg Med 18(3):288-99 [PubMed]

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