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Alain Dupuy, MD; Manuelle Viguier, MD; Christophe Bédane, MD, PhD; Florence Cordoliani, MD; Sophie Blaise, MD; Françoise Aucouturier, MD; Jean-Marie Bonnetblanc, MD; Patrice Morel, MD; Louis Dubertret, MD; Hervé Bachelez, MD, PhD

Arch Dermatol. 2004;140:91-96.

Background  Pemphigus vulgaris (PV) is a severe antibody-mediated autoimmune blistering disease. Because some patients with PV do not enter into remission, despite the use of high-dose corticosteroid therapy and immunosuppressive adjuvant treatments, new effective and safer agents are warranted to treat refractory PV. Rituximab, a monoclonal anti-CD20 antibody, induces depletion of B cells in vivo and has shown efficacy in patients with refractory antibody-mediated autoimmune disorders. We describe herein 3 patients treated with rituximab for severe PV.

Observations  Three patients with refractory PV were treated with rituximab, resulting in a clinical response in all patients, which was complete in 2 patients. A decline in titers of circulating antiepidermis autoantibodies paralleled disease activity, while circulating B cells remained undetectable for 6 to 10 months. Two patients experienced bacterial infection in the weeks following the rituximab course. A clinical relapse occurred in 2 patients, at 6 and 10 months. A second course of rituximab controlled the disease in one of them.

Conclusion  These patients' response suggests that rituximab may be a valuable treatment for refractory PV and warrants further studies to evaluate the risk-benefit ratio in patients with PV showing resistance to classic therapy.

From the Institut de Recherche sur la Peau, Institut National de la Santé et de la Recherche Médicale Unité 532, and Services de Dermatologie 1 (Drs Viguier, Dubertret, and Bachelez) et 2 (Drs Dupuy, Cordoliani, and Morel), Laboratoire d'Immunologie (Dr Aucouturier), Hôpital Saint-Louis Assistance Publique–Hôpitaux de Paris, Paris; and Service de Dermatologie, Hôpital Dupuytren, Limoges (Drs Bédane, Blaise, and Bonnetblanc), France. The authors have no relevant financial interest in this article.

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