title: Animal models to investigate pathomechanisms and evaluate novel treatments for autoimmune bullous dermatoses.
abstract: Autoimmune bullous dermatoses (AIBD), such as pemphigus, bullous pemphigoid or epidermolysis bullosa acquisita, are prototypical organ-specific autoimmune diseases. Clinically they are characterized by widespread mucocutaneous blistering, which is often difficult to treat. Patients with AIBD suffer from a significant morbidity and an increased mortality. In AIBD blistering is caused by autoantibodies targeting structural proteins of the skin. During the past decades animal models of AIBD have been developed. These animal models have greatly contributed to our current understanding of AIBD pathogenesis. Most of these insights, however, still await their translation into clinical use. Recently, AIBD animal models have been used to test the efficacy of known and novel drugs. Hence, these models are now not only employed to unravel the pathogenesis of AIBD, but also to assess therapeutic approaches to address the so far unmet high medical need for new treatments. We here review animal model of AIBD: In addition to spontaneously arising AIBD in animals, AIBD can be induced, mostly in mice, by (i) transfer of (auto)-antibodies, (ii) transfer of (auto)-antigen specific lymphocytes, (iii) immunization or (iv) by genetic modifications leading to spontaneous blistering. In combined use, these models allow dissecting all aspects of AIBD pathogenesis, i.e. loss of tolerance, autoantibody production and blistering. Overall we aim to foster a broader use of AIBD animal models, especially in translational biomedical research, to deepen our understanding of AIBD pathogenesis and to develop novel treatments for patients.
authors.forename: Hiroaki
authors.initials: H
authors.lastname: Iwata
authors.forename: Katja
authors.initials: K
authors.lastname: Bieber
authors.forename: Misa
authors.initials: M
authors.lastname: Hirose
authors.affiliation: Lubeck Institute of Experimental Dermatology, University of Lubeck, Ratzeburger Allee 160, D- 23538 Lubeck, Germany. ralf.ludwig@uksh.de.
authors.forename: Ralf J
authors.initials: RJ
authors.lastname: Ludwig
mesh_terms: D000818:Animals; D001327:Autoimmune Diseases / Q000188:drug therapy* / Q000235:genetics / Q000276:immunology / Q000473:pathology*; D004195:Disease Models, Animal; D006801:Humans; D051379:Mice; D012872:Skin Diseases, Vesiculobullous / Q000188:drug therapy* / Q000235:genetics / Q000276:immunology* / Q000473:pathology