title: Epidermolysis bullosa acquisita.
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.affiliation: Department of Dermatology, The Keck School of Medicine at the University of Southern California, Los Angeles, USA.
authors.forename: Rishu
authors.initials: R
authors.lastname: Gupta
authors.forename: David T
authors.initials: DT
authors.lastname: Woodley
authors.forename: Mei
authors.initials: M
authors.lastname: Chen
mesh_terms: D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D000818:Animals; D001323:Autoantibodies / Q000276:immunology*; D024143:Collagen Type VII / Q000276:immunology; D016572:Cyclosporine / Q000627:therapeutic use; D003622:Dapsone / Q000627:therapeutic use; D004797:Enzyme-Linked Immunosorbent Assay; D016107:Epidermolysis Bullosa Acquisita / Q000145:classification / Q000175:diagnosis* / Q000188:drug therapy* / Q000276:immunology / Q000473:pathology; D006801:Humans; D015151:Immunoblotting; D007074:Immunoglobulin G / Q000276:immunology; D007155:Immunologic Factors / Q000627:therapeutic use; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D035583:Rare Diseases; D012720:Severity of Illness Index; D012867:Skin / Q000276:immunology / Q000473:pathology
chemical_list: D000305:Adrenal Cortex Hormones; D001323:Autoantibodies; D024143:Collagen Type VII; D007074:Immunoglobulin G; D007155:Immunologic Factors; D007166:Immunosuppressive Agents; D016572:Cyclosporine; D003622:Dapsone