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Epidermolysis Bullosa Acquisita

Epidermolysis Bullosa Acquisita

Epidermolysis bullosa acquisita (EBA) is could be an uncommon, sporadic, subepithelial, mucocutaneous blistering disease that mostly establishes in adulthood. EBA is classically illustrated as a mechanobullous disorder defined by skin fragility, noninflammatory tense bullae, milia, and scarring. Alternatively, EBA can exist as an inflammatory bullous eruption remindful of bullous pemphigoid or other subepithelial autoimmune blistering diseases.

 

Panel test

o  Immunogenetics:

      Increased prevalence of HLA-DR2.

o  Immunopathology:

      DIF shows linear staining with IgG and C3 of the basement membrane, but this is not diagnostic.

      On split-skin preparations the antibody is seen to localize to the dermal side.

      IIF may also be positive.

o  Autoantibodies:

      Collagen type VII antibody, IgG

This test is helpful for initial diagnosis and disease follow up of EBA.

 

References

  1. Fine JD, Eady RA, Bauer EA, et al. The classification of inherited epidermolysis bullosa (EB): Report of the Third International Consensus Meeting on Diagnosis and Classification of EB. J Am Acad Dermatol 2008; 58:931.

Bruckner-Tuderman L, McGrath JA, Robinson EC, Uitto J. Progress in Epidermolysis bullosa research: summary of DEBRA International Research Conference 2012. J Invest Dermatol 2013; 133:2121.

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