Efficacy of rituximab in a case of refractory bullous pemphigoid

Background. - The mainstay in the treatment of bullous pemphigoid (BP) is corticosteroids. Immunosuppressive agents might be used for steroid-sparing effect. We report the case of a patient with refractory BP successfully treated with rituximab. Patients and methods. - An 83-year-old woman was hospitalized in January 2005 for severe BR She was initially treated with 30g/day of clobetasol propionate 0.05% and methotrexate (20 mg/week), with partial remission. However, every attempt to reduce topical corticosteroids resulted in a relapse of the patient's BR Subsequently, mycophenolate mofetil, azathioprine, dapsone, intravenous immunoglobulins, topical tacrolimus and systemic glucocorticoids (steroid-dependency at 20 mg/day) failed to induce complete remission. In December 2005, we decided to treat the patient with four infusions of rituximab 375 mg/m(2) at 1-week intervals, and this led to a dramatic reduction of the severity of BR In May 2006, a second course of rituximab was given. One month Later, for the first time in 18 months, complete clinical and immunological remission of BP was noted. The patient remains in complete remission, without treatment, 2 years after the last infusion of rituximab. Discussion. - The B cell-modulating effect of rituximab has encouraged its use in a variety of autoimmune diseases including pemphigus. Only five cases of refractory BP, treated with rituximab (including two paediatric cases), have so far been reported. In three of these cases, follow-up was too short to allow detection of any relapse and the other two patients had lymphocytic leukaemia requiring rituximab infusions every 2 months. In our case, the two courses of rituximab were well tolerated, induced complete clinical and immunological remission and enabled discontinuation of local and systemic corticosteroids. Conclusion. - Rituximab could offer a safe and effective therapeutic alternative for refractory BP. (C) 2009 Elsevier Masson SAS. ALL rights reserved.