[Optimizing therapy in patients with severe autoimmune blistering skin diseases]

Autoimmune bullous diseases are a heterogeneous group of disorders that can be subdivided according to the level of split formation in the intraepidermal blistering pemphigus diseases and subepidermal bullous disorders, latter including pemphigoid diseases, epidermolysis bullosa acquisita (EBA), and dermatitis herpetiformis. In the majority of autoimmune bullous disorders, disease activity can be sufficiently controlled by systemic corticosteroids in combination with further immunsuppressants/-modulants such as dapsone, doxycycline, azathioprine, mycophenolate mofetil, or methotrexate. In contrast, in pemphigus, mucous membrane pemphigoid, and EBA, treatment is challenging and conventional immunosuppressive therapy induces clinical remission only in a minority of patients. Until recently, only cyclosphosphamide and high-dose intravenous immunoglobulin (IVIG) were available as potent second-line therapies. Meanwhile, immunoadsorption and the monoclonal anti-CD20 antibody rituximab have been established as further therapeutic options. The present review focuses on efficacy, adverse events, treatment protocols, and mechanisms of action of IVIG, immunoadsorption, and rituximab in the treatment of severe and/or refractory patients with bullous autoimmune diseases.