Remission of severe autoimmune bullous disorders induced by long-term extracorporeal photochemotherapy.

Pemphigus vulgaris (PV) and epidermolysis bullosa acquisita (EBA) sometimes resist treatments. In drug-resistant cases, adjuvant treatment with extracorporeal photochemotherapy (ECP) has been reported to induce remission. However, limited numbers of patients have been reported up to date. Eleven patients with drug-resistant autoimmune bullous diseases have been treated with ECP (8 patients with PV, 3 patients with EBA). The introduction of ECP to systemic therapies of the patients with PV resulted in complete response (CR) after a limited cycle (2-6 cycles) in all, except one patient. Prednisolone doses could be tapered in all patients. ECP resulted in CR in two patients while improvement was partial in one patient with EBA after 3-6 cycles. Our patients demonstrate the efficacy of long-term ECP to be tried in the treatment of aggressive autoimmune bullous disorders. The treatment has produced a remarkable corticosteroid-sparing effect while inducing clinical remission.