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Autoimmunity to type VII collagen: epidermolysis bullosa acquisita.
Clin Rev Allergy Immunol. 2007 Oct; 33(1-2):78-84.CR

Abstract

Epidermolysis bullosa acquisita (EBA) is an acquired, autoimmune, mechanobullous disease with clinical features reminiscent of genetic dystrophic epidermolysis bullosa (DEB). EBA patients have skin fragility, blisters, scars, and milia formation. DEB is due to a genetic defect in the gene-encoding type VII collagen, which makes anchoring fibrils, structures that attach the epidermis and its underlying basement membrane zone onto the papillary dermis. DEB patients have a decrease in normally functioning anchoring fibrils. EBA patients have the same problem, but their decrease in normally functioning anchoring fibrils is because of an abnormality in their immune system in which they produce anti-type VII collagen antibodies that attack their anchoring fibrils. These IgG anti-type VII collagen antibodies are "pathogenic" because when injected into a mouse, the mouse develops an EBA-like blistering disease. EBA has several distinct clinical presentations. It can present with features similar to DEB. It can also present with features reminiscent of bullous pemphigoid, cicatricial pemphigoid, Brunsting-Perry pemphigoid, or IgA bullous dermatosis. Treatment for EBA is unsatisfactory. Some therapeutic success has been reported with colchichine, dapsone, photopheresis, infliximab, and IVIG.

Authors+Show Affiliations

Department of Dermatology, The Keck School of Medicine, University of Southern California, Los Angeles, CA, USA. dwoodley@usc.eduNo affiliation info availableNo affiliation info available

Pub Type(s)

Journal Article
Review

Language

eng

PubMed ID

18058258

Citation

Woodley, David T., et al. "Autoimmunity to Type VII Collagen: Epidermolysis Bullosa Acquisita." Clinical Reviews in Allergy & Immunology, vol. 33, no. 1-2, 2007, pp. 78-84.
Woodley DT, Remington J, Chen M. Autoimmunity to type VII collagen: epidermolysis bullosa acquisita. Clin Rev Allergy Immunol. 2007;33(1-2):78-84.
Woodley, D. T., Remington, J., & Chen, M. (2007). Autoimmunity to type VII collagen: epidermolysis bullosa acquisita. Clinical Reviews in Allergy & Immunology, 33(1-2), 78-84.
Woodley DT, Remington J, Chen M. Autoimmunity to Type VII Collagen: Epidermolysis Bullosa Acquisita. Clin Rev Allergy Immunol. 2007;33(1-2):78-84. PubMed PMID: 18058258.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Autoimmunity to type VII collagen: epidermolysis bullosa acquisita. AU - Woodley,David T, AU - Remington,Jennifer, AU - Chen,Mei, PY - 2007/12/7/pubmed PY - 2008/2/27/medline PY - 2007/12/7/entrez SP - 78 EP - 84 JF - Clinical reviews in allergy & immunology JO - Clin Rev Allergy Immunol VL - 33 IS - 1-2 N2 - Epidermolysis bullosa acquisita (EBA) is an acquired, autoimmune, mechanobullous disease with clinical features reminiscent of genetic dystrophic epidermolysis bullosa (DEB). EBA patients have skin fragility, blisters, scars, and milia formation. DEB is due to a genetic defect in the gene-encoding type VII collagen, which makes anchoring fibrils, structures that attach the epidermis and its underlying basement membrane zone onto the papillary dermis. DEB patients have a decrease in normally functioning anchoring fibrils. EBA patients have the same problem, but their decrease in normally functioning anchoring fibrils is because of an abnormality in their immune system in which they produce anti-type VII collagen antibodies that attack their anchoring fibrils. These IgG anti-type VII collagen antibodies are "pathogenic" because when injected into a mouse, the mouse develops an EBA-like blistering disease. EBA has several distinct clinical presentations. It can present with features similar to DEB. It can also present with features reminiscent of bullous pemphigoid, cicatricial pemphigoid, Brunsting-Perry pemphigoid, or IgA bullous dermatosis. Treatment for EBA is unsatisfactory. Some therapeutic success has been reported with colchichine, dapsone, photopheresis, infliximab, and IVIG. SN - 1080-0549 UR - https://www.unboundmedicine.com/medline/citation/18058258/Autoimmunity_to_type_VII_collagen:_epidermolysis_bullosa_acquisita_ L2 - https://doi.org/10.1007/s12016-007-0027-6 DB - PRIME DP - Unbound Medicine ER -