High-dose intravenous immunoglobulin (IVIG) therapy in autoimmune skin blistering diseases.

Treatment of autoimmune bullous skin diseases can often be challenging and primarily consists of systemic corticosteroids and a variety of immunosuppressants. Current treatment strategies are effective in most cases but hampered by the side effects of long-term immunosuppressive treatment. Intravenous immunoglobulin (IVIG) is one potential promising therapy for patients with autoimmune bullous skin diseases, and evidence of its effectiveness and safety is increasing. A number of autoimmune bullous skin diseases have been identified in which IVIG treatment may be beneficial. However, experience with IVIG in patients with autoimmune skin blistering disease is limited, where it is recommended for patients not responding to conventional therapy. The mode of action of IVIG in autoimmune diseases, including bullous diseases is far from being completely understood. We here summarize the clinical evidence supporting the notion, that IVIG is a promising therapeutic agent for the treatment of patients with autoimmune bullous skin disease. In addition, we review the proposed modes of action. In the future, randomized controlled trials are necessary to better determine the efficacy and adverse effects of IVIG in the treatment of autoimmune bullous skin diseases. In addition, insights into IVIG's mode of action might enable us to develop novel therapeutics to overcome the current shortage of IVIG.