Epidermolysis Bullosa Acquisita

Epidermolysis Bullosa Acquisita

 

Epidermolysis bullosa acquisita is a rare chronic autoimmune blistering disease.

Epidemiology

  • Incidence – 0.25 cases per million
  • Age of onset – all ages, peak onset age 40-50
  • Gender – M:F equal distribution

Pathology and Immunopathology

  • Subdermal blister formation
  • Linear basement membrane zone deposition of IgG in perilesional tissue by direct immunofluorescence
  • Dermal localization of serum basement membrane zone antibodies on split skin by indirect immunofluorescence

Clinical Presentation

  • Lesions of skin on areas of trauma and/or oral mucosa
  • Tense blisters with serous or hemorrhagic fluid

Diagnosis

  • Laboratory testing
    • Epithelial skin antibodies
    • IgG basement membrane zone antibodies

Differential Diagnosis

  • Bullous pemphigoid
  • Linear IgA disease
  • Porphyria cutanea tarda
  • Bullous drug reaction
  • Contact dermatitis

Disease Monitoring

  • IgG basement zone antibodies
    • IgG BP180 and BP230 antibody levels are NOT elevated as in pemphigoid; rather, the target antigen is collagen VII, accounting for the dermal staining pattern on human split skin substrate

Treatment

  • Challenging to put into remission
  • Variety of agents

See Also