Successful treatment of severe anti-p200 pemphigoid in a heart transplant recipient with a single cycle of rituximab

INTRODUCTION Anti-p200 pemphigoid is a rare autoimmune subepidermal blistering disease, first described in 1996. This disorder was initially considered as a more benign condition compared with bullous pemphigoid and epidermolysis bullosa acquisita, but in a recent series of patients, heterogeneous clinical presentations have been described, including cases with a more severe course than previously reported. We describe a case with a highly active disease in a heart transplant patient who experienced a dramatic and complete remission after 1 cycle of rituximab.