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The antibodyinduced loss of adhesion between epidermis and dermis results in blister formation and extensive erosions. There is a great need for rapidly establishing the diagnosis of these disorders since they may run a severe and potentially life\u2010threatening course. In addition, because of their rarity and heterogeneous symptoms, autoimmune bullous skin diseases often pose a major diagnostic challenge. While histopathological examinations provide evidence for the level of blister formation, immunofluorescence microscopy has been established to identify tissue\u2010bound and circulating autoantibodies. Direct immunofluorescence microscopy represents the gold standard for detecting tissue\u2010bound autoantibodies. Indirect immunofluorescence microscopy with defined tissue substrates is considered the first step in detecting circulating autoantibodies. Confirmatory tests such as ELISA, immunoblot or immunoprecipitation analyses are performed utilizing recombinant proteins or keratinocyte extracts. The later assays can be used for primary diagnosis as well as for immunoserological follow\u2010up. Systemic immunosuppressive drugs usually represent the main therapeutic regimen. Initially, systemic corticosteroids are commonly administered in combination with steroid\u2010sparing, immunosuppressive agents. Novel targeted treatments such as immunoadsorption, rituximab or high\u2010dose intravenous immunoglobulins have proven to be highly effective in severe and refractory pemphigus. This review presents a state\u2010of\u2010the\u2010art algorithm for making the diagnosis of autoimmune bullous disorders and provides an overview on currently available therapeutic options.<\/jats:p>","DOI":"10.1111\/j.1610-0387.2011.07809.x","type":"journal-article","created":{"date-parts":[[2011,10,25]],"date-time":"2011-10-25T13:39:53Z","timestamp":1319549993000},"page":"927-947","source":"Crossref","is-referenced-by-count":35,"title":["Autoimmune bullous skin diseases. 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