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
● Immunogenetics:
o Increased prevalence of HLA-DR2.
● Immunopathology:
o DIF shows linear staining with IgG and C3 of the basement membrane, but this is not diagnostic.
o On split-skin preparations the antibody is seen to localize to the dermal side.
o IIF may also be positive.
o Autoantibodies:
o Collagen type VII antibody, IgG
This test is helpful for initial diagnosis and disease follow up of EBA.
References:
1. Mayuzumi M, Akiyama M, Nishie W, et al. Childhood epidermolysis bullosa acquisita with autoantibodies against the noncollagenous 1 and 2 domains of type VII collagen: case report and review of the literature. Br J Dermatol 2006; 155:1048.
2. Iranzo P, Herrero-González JE, Mascaró-Galy JM, et al. Epidermolysis bullosa acquisita: a retrospective analysis of 12 patients evaluated in four tertiary hospitals in Spain. Br J Dermatol 2014; 171:1022.
