Pemphigus: current and future treatment strategies

Abstract

Pemphigus diseases are agroup 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 AWMFS2k 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, amonoclonal antibody directed against CD20 on Bcells, was recently approved for pemphigus vulgaris and moderate or severe pemphigus foliaceus.