Diagnosis and treatment of pemphigus

Abstract

Pemphigus is an autoimmune bullous disease, in which autoantibodies react with the cell–cell adhesion structures, desmosomes, causing blisters and erosions on the oral mucosa and skin. Pemphigus is divided into two major subtypes: pemphigus vulgaris and pemphigus foliaceus. Oral corticosteroids are the primary treatment modality for pemphigus, while other therapeutic options, such as steroid pulse therapy, immunosuppressants, intravenous immunoglobulins, plasmapheresis and anti-CD20 monoclonal antibody therapy, are occasionally employed. Immunosuppressants used to treat pemphigus include azathioprine, cyclophosphamide, cyclosporine, mycophenolate mofetil and mizoribine. In this review, we summarize the current concepts of immunotherapy for the treatment of pemphigus in the Japanese population.