Epidermolysis bullosa acquisita (EBA) is a chronic mucocutaneous autoimmune skin blistering disease.The pathogenic relevance\r\nof autoantibodies targeting type VII collagen (COL7) has been well-documented. Therefore, EBA is a prototypical autoimmune\r\ndisease with a well-characterized pathogenic relevance of autoantibody binding to the target antigen. EBA is a rare disease with an\r\nincidence of 0.2 new cases per million and per year. The current treatment of EBA relies on general immunosuppressive therapy,\r\nwhich does not lead to remission in all cases. Therefore, there is a high, so far unmet medical need for the development of novel\r\ntherapeutic options. During the last 10 years, several novel in vitro and in vivo models of EBA have been established. These models\r\ndemonstrated a critical role of the genetic background, T cells, and cytokines for mediating the loss of tolerance towards COL7.\r\nNeutrophils, complement activation, Fc gammareceptor engagement, cytokines, severalmolecules involved in cell signaling, release\r\nof reactive oxygen species, and matrix metalloproteinases are crucial for autoantibody-induced tissue injury in EBA. Based on this\r\ngrowing understanding of the diseases� pathogenesis, several potential novel therapeutic targets have emerged. In this review, the\r\nclinical presentation, pathogenesis, diagnosis, and current treatment options for EBA are discussed in detail.
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