A Case of Recalcitrant Epidermolysis Bullosa Acquisita Responsive to Rituximab Therapy

Abstract

Epidermolysis bullosa acquisita (EBA) is an autoimmune blistering disorder that occurs infrequently in children. Although typically associated with a good prognosis in children, there are rare cases that are refractory to treatment with conventional immunosuppressive therapy. Rituximab, an anti-CD20 monoclonal antibody, has been reported to be effective in the adult form of EBA. We report a case of a child with severe and recalcitrant EBA exhibiting a sustained response to treatment with rituximab.