A Case Report of the Use of Rituximab and the Epidermolysis Bullosa Disease Activity Scoring Index (EBDASI) in a Patient with Epidermolysis Bullosa Acquisita with Extensive Esophageal Involvement.

A 49-year-old man with recalcitrant mechanobullous epidermolysis bullosa acquisita (EBA) with significant esophageal involvement was treated with rituximab. EBA is a chronic autoimmune subepidermal bullous disease. It is characterized by skin fragility and scarring caused by circulating and tissue bound antibodies to type VII collagen. EBA is often unresponsive or poorly responsive to conventional immunosuppressive therapies such as corticosteroids, methotrexate, and cyclosporine. The burden of long-term use of immunosuppressants also limits their use in the treatment of chronic autoimmune diseases such as EBA. Since a validated and objective way of measuring disease activity in patients with EBA has not been described, we used the Epidermolysis Bullosa Disease Activity Scoring Index (EBDASI), for hereditary EB, as a surrogate to measure disease severity and activity in our patient with EBA. After three courses of rituximab over three years, our patient has achieved near complete clinical remission from disease activity. The patient's response suggests that treatment with rituximab may be a valuable treatment regimen for severe mechanobullous EBA, which is demonstrated by paralleled declines in objective disease activity scores, the EBDASI. This is in line with recently observed beneficial effects of rituximab in the management of EBA.