title: Immune mechanism-targeted treatment of experimental epidermolysis bullosa acquisita.
abstract: Epidermolysis bullosa acquisita (EBA) is an autoimmune bullous dermatosis characterized by chronic mucocutaneous blistering caused by autoantibodies directed against type VII collagen. EBA causes a high morbidity and is difficult to treat. Model systems have significantly broadened our understanding of EBA pathogenesis, leading to the identification of numerous therapeutic targets. Of these, so far, a few have been evaluated for their therapeutic potential in preclinical models. In mice, EBA can be induced by transfer of anti-type VII collagen antibodies or by immunization with the protein. The latter model, immunization-induced EBA, is ideal to test drugs for their therapeutic efficacy. Here, mice with already established disease can be treated for prolonged periods. Albeit time consuming, results from immunization-induced EBA will pave the way for clinical application in patients. As the key pathogenic principle, that is, autoantibody-induced, leukocyte-mediated tissue injury and inflammation, is shared by other diseases, these findings may have translational applications beyond EBA.
authors.affiliation: a University of Luebeck, Luebeck Institute of Experimental Dermatology, Ratzeburger Allee 160, Luebeck, Germany .
authors.forename: Ralf
authors.initials: R
authors.lastname: Ludwig
mesh_terms: D000818:Animals; D001323:Autoantibodies / Q000276:immunology*; D024141:Collagen Type IV / Q000276:immunology*; D004195:Disease Models, Animal; D016107:Epidermolysis Bullosa Acquisita / Q000276:immunology / Q000473:pathology / Q000628:therapy; D006801:Humans; D051379:Mice
chemical_list: D001323:Autoantibodies; D024141:Collagen Type IV
keywords: GM-CSF; PI3K; anakinra; animal models; cell signaling; cytokines; epidermolysis bullosa acquisita; p38 MAPK; treatment