Treatment of autoimmune mucocutaneous blistering diseases with intravenous immunoglobulin therapy

Abstract

Autoimmune mucocutaneous blistering diseases (AMBDs) are a group of rare diseases that affect the skin and mucous membranes and are potentially fatal. They have variable clinical presentation, course and prognosis. Their immunopathology is well-characterised and target antigens have been studied. Many patients respond to conventional therapy, which consists of high-dose long-term systemic corticosteroids with an immunosuppressive agent, but side effects develop that can produce a poor quality of life. Many patients develop significant sequelae, such as blindness, loss of voice, vaginal and anal stenosis. In most patients cause of death is opportunistic infections secondary to immune suppression. To date, intravenous immunoglobulin (IVIg) has been reported to have benefited 156 patients with AMBDs. Its most important features include the ability to reduce or eliminate conventional therapy, the enabling of clinical control, the ability to induce and maintain long-term clinical remission, the capacity for usage based on a defined protocol with a described end point and a resulting increase in quality of life. IVIg produces the best clinical outcome when combined with aggressive topical therapy, sublesional injections of triamcinolone and rapid detection, and early treatment of cutaneous and mucosal infection. Successful therapy requires a physician to spend significant time with each patient. This manuscript provides the opinion of the author on the current use of IVIg to treat AMBDs.

• blistering diseases
• conventional immunosuppressive therapies
• epidermolysis bullosa acquisita
• IVIg therapy
• pemphigoid
• pemphigus
• pharmacoeconomics
• protocol for IVIg
• supportive therapies with IVIg