Background Mucous membrane pemphigoid (MMP) is an autoimmune blistering disease. In patients who do not respond to conventional therapy, rituximab (RTX) may be an option. The current literature on ...the treatment of MMP with RTX is limited. Objective In this review, the data on 28 patients with MMP treated with RTX are critically analyzed. The goal is to provide objective information useful in decision making and treatment. Methods A PubMed search using the key words “rituximab” and “mucous membrane pemphigoid” was made in the English language only. The studies were divided into case reports and case series. Results In the final analysis, 20 of 28 patients had a complete response, 3 had a partial response, 2 were nonresponders, and 1 had stabilization of disease. In 1 patient, stabilization of upper airway disease was observed but the patient developed bilateral blindness as a result of progression of disease. Hence, the patient was considered a treatment failure. One died from infection. At least half of the patients were treated with a second cycle because of relapse or lack of response. Limitations Long-term follow-up after RTX therapy is lacking. Hence, the clinical benefit of inducing long-term remissions cannot be assessed. Responses of individual mucosal sites cannot be differentiated. Studies on B-cell levels and antibody responses are lacking. Conclusion Using the protocol described, RTX benefits patients with recalcitrant MMP. Some patients fail treatment or experience a relapse. The ability of RTX to influence the clinical course of MMP remains to be determined.
