title: Epidermolysis Bullosa Acquisita: From Pathophysiology to Novel Therapeutic Options.
abstract: Epidermolysis bullosa acquisita (EBA) is a prototypic organ-specific autoimmune disease induced by autoantibodies to type VII collagen causing mucocutaneous blisters. In the inflammatory (bullous pemphigoid-like) EBA variant, autoantibody binding is followed by a lesional inflammatory cell infiltration, and the overall clinical picture may be indistinguishable from that of bullous pemphigoid, the latter being the most common autoimmune bullous disease. The last decade witnessed the development of several mouse models of inflammatory EBA that facilitated the elucidation of the pathogenesis of autoantibody-induced, cell-mediated subepidermal blistering diseases and identified new therapeutic targets for these and possibly other autoantibody-driven disorders.
authors.affiliation: Department of Dermatology, University of Lübeck, Lübeck, Germany. Electronic address: Michael.Kasperkiewicz@uk-sh.de.
authors.forename: Michael
authors.initials: M
authors.lastname: Kasperkiewicz
authors.affiliation: Department of Dermatology, University of Lübeck, Lübeck, Germany.
authors.forename: Christian D
authors.initials: CD
authors.lastname: Sadik
authors.affiliation: Lübeck Institute of Experimental Dermatology, University of Lübeck, Lübeck, Germany.
authors.forename: Katja
authors.initials: K
authors.lastname: Bieber
authors.affiliation: Department of Dermatology, University of Lübeck, Lübeck, Germany; Lübeck Institute of Experimental Dermatology, University of Lübeck, Lübeck, Germany.
authors.forename: Saleh M
authors.initials: SM
authors.lastname: Ibrahim
authors.affiliation: Institute for Systemic Inflammation Research, University of Lübeck, Lübeck, Germany.
authors.forename: Rudolf A
authors.initials: RA
authors.lastname: Manz
authors.affiliation: Department of Dermatology, University of Lübeck, Lübeck, Germany; Lübeck Institute of Experimental Dermatology, University of Lübeck, Lübeck, Germany.
authors.forename: Enno
authors.initials: E
authors.lastname: Schmidt
authors.affiliation: Department of Dermatology, University of Lübeck, Lübeck, Germany; Lübeck Institute of Experimental Dermatology, University of Lübeck, Lübeck, Germany.
authors.forename: Detlef
authors.initials: D
authors.lastname: Zillikens
authors.affiliation: Department of Dermatology, University of Lübeck, Lübeck, Germany; Lübeck Institute of Experimental Dermatology, University of Lübeck, Lübeck, Germany.
authors.forename: Ralf J
authors.initials: RJ
authors.lastname: Ludwig
mesh_terms: D000818:Animals; D000911:Antibodies, Monoclonal / Q000627:therapeutic use; D001323:Autoantibodies / Q000276:immunology*; D001327:Autoimmune Diseases / Q000188:drug therapy / Q000473:pathology*; D004195:Disease Models, Animal; D016107:Epidermolysis Bullosa Acquisita / Q000276:immunology* / Q000503:physiopathology; D005260:Female; D006801:Humans; D007111:Immunity, Cellular / Q000502:physiology*; D007167:Immunotherapy / Q000379:methods; D008297:Male; D051379:Mice; D011379:Prognosis; D018570:Risk Assessment; D016896:Treatment Outcome
chemical_list: D000911:Antibodies, Monoclonal; D001323:Autoantibodies