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Epidermolysis bullosa acquisita: autoimmunity to anchoring fibril collagen.
Autoimmunity. 2012 Feb; 45(1):91-101.A

Abstract

Epidermolysis bullosa acquisita (EBA) is a rare and acquired autoimmune subepidermal bullous disease of skin and mucosa. EBA includes various distinct clinical manifestations resembling genetic dystrophic epidermolysis bullosa (DEB), Bullous pemphigus, Brunsting-Perry pemphigoid, or cicatricial pemphigoid. These patients have autoantibodies against type VII collagen (C7), an integral component of anchoring fibrils (AFs), which are responsible for attaching the dermis to the epidermis. Destruction or perturbation of the normal functioning AFs clinically results in skin fragility, blisters, erosions, scars, milia, and nail loss, all features reminiscent of genetic dystrophic epidermolysis bullosa. These anti-C7 antibodies are "pathogenic" because when injected into a mouse, the mouse develops an EBA-like blistering disease. Currently, treatment is often unsatisfactory; however, some success has been achieved with colchicine, dapsone, photopheresis, plasmapheresis, infliximab, rituximab, and IVIG.

Authors+Show Affiliations

Department of Dermatology, The Keck School of Medicine at the University of Southern California, Los Angeles, CA 90033, USA.No affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

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

Language

eng

PubMed ID

21955050

Citation

Chen, Mei, et al. "Epidermolysis Bullosa Acquisita: Autoimmunity to Anchoring Fibril Collagen." Autoimmunity, vol. 45, no. 1, 2012, pp. 91-101.
Chen M, Kim GH, Prakash L, et al. Epidermolysis bullosa acquisita: autoimmunity to anchoring fibril collagen. Autoimmunity. 2012;45(1):91-101.
Chen, M., Kim, G. H., Prakash, L., & Woodley, D. T. (2012). Epidermolysis bullosa acquisita: autoimmunity to anchoring fibril collagen. Autoimmunity, 45(1), 91-101. https://doi.org/10.3109/08916934.2011.606450
Chen M, et al. Epidermolysis Bullosa Acquisita: Autoimmunity to Anchoring Fibril Collagen. Autoimmunity. 2012;45(1):91-101. PubMed PMID: 21955050.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Epidermolysis bullosa acquisita: autoimmunity to anchoring fibril collagen. AU - Chen,Mei, AU - Kim,Gene H, AU - Prakash,Lori, AU - Woodley,David T, Y1 - 2011/09/28/ PY - 2011/9/30/entrez PY - 2011/10/1/pubmed PY - 2012/5/25/medline SP - 91 EP - 101 JF - Autoimmunity JO - Autoimmunity VL - 45 IS - 1 N2 - Epidermolysis bullosa acquisita (EBA) is a rare and acquired autoimmune subepidermal bullous disease of skin and mucosa. EBA includes various distinct clinical manifestations resembling genetic dystrophic epidermolysis bullosa (DEB), Bullous pemphigus, Brunsting-Perry pemphigoid, or cicatricial pemphigoid. These patients have autoantibodies against type VII collagen (C7), an integral component of anchoring fibrils (AFs), which are responsible for attaching the dermis to the epidermis. Destruction or perturbation of the normal functioning AFs clinically results in skin fragility, blisters, erosions, scars, milia, and nail loss, all features reminiscent of genetic dystrophic epidermolysis bullosa. These anti-C7 antibodies are "pathogenic" because when injected into a mouse, the mouse develops an EBA-like blistering disease. Currently, treatment is often unsatisfactory; however, some success has been achieved with colchicine, dapsone, photopheresis, plasmapheresis, infliximab, rituximab, and IVIG. SN - 1607-842X UR - https://www.unboundmedicine.com/medline/citation/21955050/Epidermolysis_bullosa_acquisita:_autoimmunity_to_anchoring_fibril_collagen_ L2 - https://www.tandfonline.com/doi/full/10.3109/08916934.2011.606450 DB - PRIME DP - Unbound Medicine ER -