Pemphigus: current and future treatment strategies

Pemphigus diseases are a group of organ-specific autoimmune diseases which are characterised by the production of autoantibodies against intra-epidermal adhesion molecules and structural proteins of skin and mucosae. Depending on the entity, patients develop blisters and erosions on the skin and/or mucosae. According to the AWMF S2k guidelines for diagnosis and therapy of pemphigus diseases, a systemic therapy is recommended. Initially, high-dose, oral corticosteroids in combination with immunosuppressive drugs as corticosteroid-sparing agent, usually azathioprine or mycophenolate mofetil, can be used. Furthermore, rituximab, a monoclonal antibody directed against CD20 on B cells, was recently approved for pemphigus vulgaris and moderate or severe pemphigus foliaceus.