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Epidermolysis bullosa acquisita.
Clin Dermatol. 2012 Jan-Feb; 30(1):60-9.CD

Abstract

Epidermolysis bullosa acquisita (EBA) is a rare, acquired, chronic subepidermal bullous disease of the skin and mucosa characterized by autoantibodies to type VII collagen (C7) structures, a major component of anchoring fibrils, which attach the epidermis to the dermis. EBA patients have tissue-bound and circulating antitype C7 autoantibodies that attack type C7 and result in a reduction or perturbation of normally functioning anchoring fibrils. Patients with EBA have skin fragility, blisters, erosions, scars, milia, and nail loss, all features reminiscent of genetic dystrophic epidermolysis bullosa. These immunoglobulin G antitype C7 antibodies are pathogenic, because when they are injected into mice, the mice develop an EBA-like blistering disease. In addition to the classical mechanobullous presentation, EBA also has several other distinct clinical syndromes similar to bullous pemphigoid, Brunsting-Perry pemphigoid, or cicatricial pemphigoid. Although treatment for EBA is often unsatisfactory, some therapeutic success has been achieved with colchicine, dapsone, plasmapheresis, photopheresis, infliximab, and intravenous immunoglobulin.

Authors+Show Affiliations

Department of Dermatology, The Keck School of Medicine at the University of Southern California, Los Angeles, USA.No affiliation info availableNo affiliation info available

Pub Type(s)

Journal Article
Research Support, N.I.H., Extramural
Review

Language

eng

PubMed ID

22137228

Citation

Gupta, Rishu, et al. "Epidermolysis Bullosa Acquisita." Clinics in Dermatology, vol. 30, no. 1, 2012, pp. 60-9.
Gupta R, Woodley DT, Chen M. Epidermolysis bullosa acquisita. Clin Dermatol. 2012;30(1):60-9.
Gupta, R., Woodley, D. T., & Chen, M. (2012). Epidermolysis bullosa acquisita. Clinics in Dermatology, 30(1), 60-9. https://doi.org/10.1016/j.clindermatol.2011.03.011
Gupta R, Woodley DT, Chen M. Epidermolysis Bullosa Acquisita. Clin Dermatol. 2012 Jan-Feb;30(1):60-9. PubMed PMID: 22137228.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Epidermolysis bullosa acquisita. AU - Gupta,Rishu, AU - Woodley,David T, AU - Chen,Mei, PY - 2011/12/6/entrez PY - 2011/12/6/pubmed PY - 2012/3/31/medline SP - 60 EP - 9 JF - Clinics in dermatology JO - Clin Dermatol VL - 30 IS - 1 N2 - Epidermolysis bullosa acquisita (EBA) is a rare, acquired, chronic subepidermal bullous disease of the skin and mucosa characterized by autoantibodies to type VII collagen (C7) structures, a major component of anchoring fibrils, which attach the epidermis to the dermis. EBA patients have tissue-bound and circulating antitype C7 autoantibodies that attack type C7 and result in a reduction or perturbation of normally functioning anchoring fibrils. Patients with EBA have skin fragility, blisters, erosions, scars, milia, and nail loss, all features reminiscent of genetic dystrophic epidermolysis bullosa. These immunoglobulin G antitype C7 antibodies are pathogenic, because when they are injected into mice, the mice develop an EBA-like blistering disease. In addition to the classical mechanobullous presentation, EBA also has several other distinct clinical syndromes similar to bullous pemphigoid, Brunsting-Perry pemphigoid, or cicatricial pemphigoid. Although treatment for EBA is often unsatisfactory, some therapeutic success has been achieved with colchicine, dapsone, plasmapheresis, photopheresis, infliximab, and intravenous immunoglobulin. SN - 1879-1131 UR - https://www.unboundmedicine.com/medline/citation/22137228/Epidermolysis_bullosa_acquisita_ L2 - https://linkinghub.elsevier.com/retrieve/pii/S0738-081X(11)00087-3 DB - PRIME DP - Unbound Medicine ER -