Short-time extracorporeal photochemotherapy in the treatment of drug-resistant autoimmune bullous diseases

Background Bullous pemphigoid (BP) and pemphigus vulgaris (PV) are potentia lly severe diseases. In drug-resistant PV and pemphigus foliaceus, long-ter m adjuvant treatment with extracorporeal photochemotherapy (photopheresis, ECP) has been reported to induce remission. Only limited numbers of patient s have been reported so far. No information about the effectiveness in drug -resistant BP is available, Patients and Methods: Seven patients with drug- resistant autoimmune bullous diseases have been referred to the photopheres is center of Jena (3 x PV, 3 x BP, 1 x pemphigus foliaceus). The age ranged from 31 to 85 years. ECP was performed on 2 consecutive days once a month. Oral 8-methoxypsoralen was used as photosensitizer, Previous immunosuppres sive treatment with either prednisolone or prednisolone/azathioprine was co ntinued. Results: Complete remission (absence of skin or mucous membrane le sions) was achieved in the 6 patients with PV and BP after 1-4 cycles. In t he patient suffering from pemphigus foliaceus, a partial remission (>50% im provement) was observed; in all except this patient, the immunosuppressive treatment could be tapered. Long-term remission was achieved. No severe sid e effects were observed. The treatment was well tolerated. Conclusions: Sho rt-time ECP is an effective and safe adjuvant treatment for patients with d rug-resistant autoimmune bullous diseases. It can induce remission and allo ws dose tapering of the immunosuppressive drugs.