Emerging treatment for epidermolysis bullosa acquisita

Abstract

Epidermolysis bullosa acquisita (EBA) is a rare, chronic, subepidermal, mucocutaneous blistering disease characterized by skin fragility and spontaneous as well as trauma-induced blisters that heal with scar formation and milia. Treatment is often frustrating because conventional therapy with corticosteroids and immunosuppressive agents frequently does not result in significant clinical improvement. We review the conventional treatment of EBA and critically analyze the literature on various adjuvants and therapeutic modalities that have recently been used. These include cyclosporine, colchicine, plasmapheresis, extracorporeal photochemotherapy, and intravenous gammaglobulins. Although the data are preliminary, they suggest that intravenous immunoglobulins may be a promising treatment modality for resistant, nonresponsive, or refractory EBA. The use of intravenous immunoglobulins results in significant improvement of skin and mucosal lesions, and it is quite safe, with minimal side effects. (J Am Acad Dermatol 2001;44:818-28.)