Immunoadsorption in the treatment of pemphigus.

The autoimmune bullous skin disorder pemphigus is characterized by the presence of autoantibodies that target distinct adhesion molecules of the epidermis. The autoantibodies result in a loss of the epidermal adhesive properties, which in turn leads to the appearance of blisters and erosions on skin and mucosal surfaces. In the majority of cases, disease activity can be sufficiently controlled by systemic corticosteroids in combination with immunsuppressants, but pemphigus often is recalcitrant to conventional immunosuppressive therapy. Clinical remission can be obtained only in a minority of patients. In the past, only cyclophosphamide and high-dose intravenous immunoglobulin (IVIG) were available as second-line therapies. Meanwhile, immunoadsorption and monoclonal anti-CD 20 antibody rituximab have been established as further therapeutic options.