title: Epidermolysis bullosa acquisita: autoimmunity to anchoring fibril collagen.
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.affiliation: Department of Dermatology, The Keck School of Medicine at the University of Southern California, Los Angeles, CA 90033, USA.
authors.forename: Mei
authors.initials: M
authors.lastname: Chen
authors.forename: Gene H
authors.initials: GH
authors.lastname: Kim
authors.forename: Lori
authors.initials: L
authors.lastname: Prakash
authors.forename: David T
authors.initials: DT
authors.lastname: Woodley
mesh_terms: D000818:Animals; D001327:Autoimmune Diseases / Q000276:immunology*; D015551:Autoimmunity / Q000276:immunology*; D024143:Collagen Type VII / Q000276:immunology*; D016107:Epidermolysis Bullosa Acquisita / Q000175:diagnosis / Q000276:immunology* / Q000628:therapy; D006801:Humans
chemical_list: D024143:Collagen Type VII