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epidermolysis bullosa acquisita (EBA)
Etiology:
- autoimmune
- in some patients associated with
a) systemic lupus erythematosus
b) inflammatory bowel disease
- mechanically-induced
Epidemiology:
- rare
Pathology:
- blistering occurs in dermis
- subepidermal cleavage without acantholysis
- IgG deposition at the basement membrane
- autoantibodies to collagen type 7
* histopathology images [4]
Clinical manifestations:
- mechanically-induced bullae & erosions mostly on extensor surfaces
- lesions heal with scarring & milia
* images [5]
Laboratory:
- skin biopsy with direct immunofluorescence microscopy [1]
- see ARUP consult [2]
Interactions
disease interactions
General
autoimmune bullous disease (... pemphigus, bullous pemphigoid)
epidermolysis bullosa
genetic disease of the skin (genodermatosis)
References
- Medical Knowledge Self Assessment Program (MKSAP) 15, 16, 17, 18.
American College of Physicians, Philadelphia 2009, 2012, 2015, 2018,
- ARUP Consult: Epidermolysis Bullosa Acquisita
The Physician's Guide to Laboratory Test Selection & Interpretation
https://www.arupconsult.com/content/epidermolysis-bullosa-acquisita
- Reddy H, Shipman AR, Wojnarowska F.
Epidermolysis bullosa acquisita and inflammatory bowel disease:
a review of the literature.
Clin Exp Dermatol. 2013 Apr;38(3):225-9
PMID: 23517353
- Rao J, Elston DM (histopathology images)
Medscape: Epidermolysis Bullosa Acquisita
http://emedicine.medscape.com/article/1063083-overview
- DermNet NZ. Epidermolysis bullosa acquisita (images)
http://www.dermnetnz.org/immune/epidermolysis-bullosa-acquisita.html