Current Therapy in Autoimmune Bullous Diseases

Abstract

The goal of the treatment of autoimmune bullous disease is to reduce the production of pathogenic autoantibodies or increase elimination of pathogenic autoantibodies from serum of the patients. Immunosuppressive therapy reduces the production of autoanti-bodies. The therapy protocol is divided into three phases. The first is a control phase with the highest dose of immunosuppressive drugs suppressing activity of disease, followed with a consolidation phase, when the bulk of lesions is healed. The last phase is a maintenance phase when immunosuppressive medication is gradually tapered to the lowest level that suppresses the appearance of new lesions. In complete remission off therapy, the patient reached complete clinical remission and does not use any immuno-suppressive medication. In complete remission on therapy, the patient uses a minimal immunosuppressive therapy. The first-line treatment is corticosteroids in pemphigus and pemphigoid groups. Adjuvant immunosuppressive drugs are combined with sys-temic corticosteroids and display a corticosteroid sparing effect. First-line immunosup-pressive adjuvants comprise azathioprine, mycophenolate mofetil and mycophenolic acid. Rituximab, intravenous immunoglobulin G, immunoadsorption, cyclophospha-mide, dapsone, and methotrexate are regarded as the second-line adjuvants. In derma-titis herpetiformis, a gluten free diet eliminates the clinical symptoms. Dapsone is regarded to be a valid therapeutic option in management of dermatitis herpetiformis.