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Autoimmunity to type VII collagen: epidermolysis bullosa acquisita.
Curr Dir Autoimmun. 2008; 10:195-205.CD

Abstract

Epidermolysis bullosa acquisita (EBA) is an acquired, mechanobullous disease characterized by autoimmunity to type VII collagen. Type VII collagen makes anchoring fibrils, structures that connect the epidermis and its underlying basement membrane zone to the papillary dermis. EBA patients exhibit skin fragility, blisters, scars and milia formation reminiscent of genetic dystrophic epidermolysis bullosa (DEB). DEB patients have diminutive or absent anchoring fibrils due to a genetic defect in the gene encoding type VII collagen. EBA patients have a decrease in normally functioning anchoring fibrils secondary to an abnormality in their immune system in which they produce 'pathogenic' IgG anti-type VII collagen antibodies. The pathogenicity of these autoantibodies has been demonstrated by passive transfer animal models, in which anti-type VII collagen antibodies injected into a mouse produced an EBA-like blistering disease in the animal. EBA has several distinct clinical presentations. It can present with features similar to DEB, bullous pemphigoid, cicatricial pemphigoid, Brunsting-Perry pemphigoid or IgA bullous dermatosis. Treatment for EBA is unsatisfactory, however, some therapeutic success has been reported with colchicine, dapsone, photophoresis, infliximab and intravenous immunoglobulin.

Authors+Show Affiliations

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

Pub Type(s)

Journal Article
Review

Language

eng

PubMed ID

18460887

Citation

Remington, Jennifer, et al. "Autoimmunity to Type VII Collagen: Epidermolysis Bullosa Acquisita." Current Directions in Autoimmunity, vol. 10, 2008, pp. 195-205.
Remington J, Chen M, Burnett J, et al. Autoimmunity to type VII collagen: epidermolysis bullosa acquisita. Curr Dir Autoimmun. 2008;10:195-205.
Remington, J., Chen, M., Burnett, J., & Woodley, D. T. (2008). Autoimmunity to type VII collagen: epidermolysis bullosa acquisita. Current Directions in Autoimmunity, 10, 195-205. https://doi.org/10.1159/000131455
Remington J, et al. Autoimmunity to Type VII Collagen: Epidermolysis Bullosa Acquisita. Curr Dir Autoimmun. 2008;10:195-205. PubMed PMID: 18460887.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Autoimmunity to type VII collagen: epidermolysis bullosa acquisita. AU - Remington,Jennifer, AU - Chen,Mei, AU - Burnett,Julie, AU - Woodley,David T, PY - 2008/5/8/pubmed PY - 2008/8/13/medline PY - 2008/5/8/entrez SP - 195 EP - 205 JF - Current directions in autoimmunity JO - Curr Dir Autoimmun VL - 10 N2 - Epidermolysis bullosa acquisita (EBA) is an acquired, mechanobullous disease characterized by autoimmunity to type VII collagen. Type VII collagen makes anchoring fibrils, structures that connect the epidermis and its underlying basement membrane zone to the papillary dermis. EBA patients exhibit skin fragility, blisters, scars and milia formation reminiscent of genetic dystrophic epidermolysis bullosa (DEB). DEB patients have diminutive or absent anchoring fibrils due to a genetic defect in the gene encoding type VII collagen. EBA patients have a decrease in normally functioning anchoring fibrils secondary to an abnormality in their immune system in which they produce 'pathogenic' IgG anti-type VII collagen antibodies. The pathogenicity of these autoantibodies has been demonstrated by passive transfer animal models, in which anti-type VII collagen antibodies injected into a mouse produced an EBA-like blistering disease in the animal. EBA has several distinct clinical presentations. It can present with features similar to DEB, bullous pemphigoid, cicatricial pemphigoid, Brunsting-Perry pemphigoid or IgA bullous dermatosis. Treatment for EBA is unsatisfactory, however, some therapeutic success has been reported with colchicine, dapsone, photophoresis, infliximab and intravenous immunoglobulin. SN - 1422-2132 UR - https://www.unboundmedicine.com/medline/citation/18460887/Autoimmunity_to_type_VII_collagen:_epidermolysis_bullosa_acquisita_ L2 - https://www.karger.com?DOI=10.1159/000131455 DB - PRIME DP - Unbound Medicine ER -