Successful treatment of recalcitrant cicatricial pemphigoid with a combination of plasma exchange and cyclophosphamide

We describe two patients with severe oral cicatricial pemphigoid, one of whom also had severe pharyngeal involvement. Both patients were resistant to treatment with corticosteroids and other standard immunosuppressive therapies. Plasma exchange alone proved to be only temporarily effective, but the combination of plasma exchange with subsequent cyclophosphamide resulted in a remission in both patients. Both patients experienced mild side-effects during the plasma exchange treatment (urticaria and mild hypotension). At present, at follow-up of 6 and 9 years, respectively, the patients have no symptoms of active disease and have not required any further immunosuppressive treatment.