Prolonged Treatment with Rituximab in Patients with Refractory Pemphigus Vulgaris

Objectives: Recent reports have documented swift responses in refractory pemphigus vulgaris to rituximab, a monoclonal anti-CD20 antibody. Nevertheless, no standard protocol has yet been established for the administration of rituximab in patients with pemphigus vulgaris. We report the results of prolonged treatment with rituximab therapy in two patients with refractory pemphigus vulgaris.

Methods: A 22- and a 27-year-old man were diagnosed with pemphigus vulgaris according to clinical, histopathological and immunofluorescence criteria. They failed to respond after several years of steroid and immunosuppressive treatment and experienced adverse effects. Rituximab was administered intravenously at a dose of 375 mg/m2 once weekly for 4 weeks.

Results: Single infusions of rituximab 375 mg/m2 were repeated at 2-monthly intervals four times because of a delayed response. The treatment was well tolerated, no side effects were observed and the previous corticosteroid therapy could be progressively withdrawn.

Conclusions: The good response of our patients suggests that rituximab may be a valuable treatment option for refractory pemphigus vulgaris. It is important that clinicians are aware that the response to rituximab in pemphigus may be delayed, and that prolonged treatment may have a good outcome.