epidermolysis bullosa acquisita (EBA)
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Etiology
- autoimmune
- in some patients associated with
- systemic lupus erythematosus
- 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]
More general terms
- epidermolysis bullosa
- genetic disease of the skin (genodermatosis)
- autoimmune bullous disease (... pemphigus, bullous pemphigoid)
References
- ↑ Jump up to: 1.0 1.1 Medical Knowledge Self Assessment Program (MKSAP) 15, 16, 17, 18. American College of Physicians, Philadelphia 2009, 2012, 2015, 2018,
- ↑ Jump up to: 2.0 2.1 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: https://www.ncbi.nlm.nih.gov/pubmed/23517353
- ↑ Jump up to: 4.0 4.1 Rao J, Elston DM (histopathology images) Medscape: Epidermolysis Bullosa Acquisita http://emedicine.medscape.com/article/1063083-overview
- ↑ Jump up to: 5.0 5.1 DermNet NZ. Epidermolysis bullosa acquisita (images) http://www.dermnetnz.org/immune/epidermolysis-bullosa-acquisita.html