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Report of 12 Cases and a Review of the Literature

Giuseppe Cianchini, MD; Rosamaria Corona, DSc, MD; Alessandra Frezzolini, BSc; Marina Ruffelli, BSc; Biagio Didona, MD; Pietro Puddu, MD

Arch Dermatol. 2007;143(8):1033-1038.

Background  Treatment of pemphigus vulgaris can be challenging. Systemic steroids associated with other immunosuppressant agents are the mainstay of therapy and have dramatically reduced morbidity and mortality from pemphigus vulgaris. In some patients, however, these agents are not able to control the disease or have severe adverse effects. Rituximab (MabThera; Roche, Basel, Switzerland), a chimeric monoclonal anti-CD20 antibody, induces depletion of B cells in vivo and has shown efficacy in patients with refractory antibody-mediated autoimmune disorders. We report 10 cases of pemphigus vulgaris and 2 cases of pemphigus foliaceous treated with rituximab—to our knowledge the largest series of patients so far—and review the existing literature on the topic.

Observation  The 12 patients were selected for treatment with the anti-CD20 antibody. Rituximab was administered intravenously at a dosage of 375 mg/m² once weekly for 4 weeks. The treatment was well tolerated, and all 12 patients showed a good clinical response during an 18-month follow-up period, along with a consensual decline of the serum antidesmoglein titers. No infectious complications were observed.

Conclusions  Rituximab is able to induce a prolonged clinical remission in patients with both pemphigus vulgaris and pemphigus foliaceous after a single course of 4 treatments. The preliminary experiences worldwide make rituximab a promising therapeutic option for patients with autoimmune diseases. The high costs and the limited knowledge of long-term adverse effects, however, limit its use to selected patients with treatment-resistant or life-threatening disease.

Author Affiliations: Division of Dermatology (Drs Cianchini, Corona, Didona, and Puddu) and Allergology and Immunology Laboratory (Mss Frezzolini and Ruffelli), Istituto Dermopatico dell’Immacolata, Istituto di Ricovero e Cura a Carrattere Scientifico, Rome, Italy.