Rituximab for the treatment of corticosteroid-refractory pemphigus vulgaris with oral and skin manifestations.

摘要

Pemphigus vulgaris (PV) is a life-threatening autoimmune blistering disease affecting the skin and mucosa and is associated with increased morbidity and mortality. Once the diagnosis is established, the main stay of treatment of PV is with systemic corticosteroids to control the disease and then to consolidate the management with other immunosuppressive agents. A small group of patients with severe pemphigus, however, remain relcalcitrant to both steroids and azathioprine, and disease is difficult to control. When considering the treatment of refractory PV, there is increasing evidence for the successful use of the monoclonal anti-CD20 antibody, rituximab. We report here six cases of patients presenting with oral and skin PV with recalcitrant or rapidly progressive disease treated with a novel dosing regimen of rituximab as a single agent. All patients achieved a complete response to a maximum follow-up of 34 months.