Pemphigoid diseases (PDs) are a group of autoimmune bullous diseases characterized and caused by autoantibodies targeting structural proteins of the skin and mucous membranes. Chronic inflammation, subepidermal blistering, and often scaring are the clinical characteristics of PDs. Itching and, in severe cases, disabilities resulting from scaring (i.e., blindness, esophageal strictures) are the leading subjective symptoms. Treatment of PDs, which is based on nonspecific immunosuppression, is challenging because of frequent relapses, lack of efficacy, and numerous adverse events. In addition, the incidence of PDs is increasing. Given the high morbidity, limited therapeutic options, and increasing incidence, there is a growing urgency for drug discovery to help treat this condition. The recent development of PD model systems has added to the understanding of PD pathogenesis and, based on these insights, new clinical trials will soon be launched. The (auto-)antibody transfer PD models allow for investigations into autoantibody-mediated tissue pathology, while immunization-induced PD models more closely resemble the clinical situation. The latter duplicate all aspects of the human disease and are useful for investigating PD pathogenesis and testing therapeutic interventions. This article describes antibody transfer and immunization-induced PD mouse models currently employed for translational PD research. © 2019 by John Wiley & Sons, Inc.
Keywords: animal models; cytokine; pemphigoid disease; pre-clinical model systems; signaling; therapy.
© 2019 John Wiley & Sons, Inc.