Pemphigus vulgaris is a potentially fatal autoimmune mucocutaneous blistering disease. Conventional therapy consists of high-dose corticosteroids, immunosuppressive agents, and intravenous immune globulin. More recently newer agenst, such as Cellcept and Dapsone. Among the new approaches is Rituxan. A number of case reprots suggest that Rituxan is an effective reatment but it had not been prospectively studies.For example, a recent report of 11 pateitns treated with Rituxan and IVIG, showed that of 11 patients, 9 had rapid resolution of lesions and a clinical remission lasting 22 to 37 months (mean, 31.1). All immunosuppressive therapy, including prednisone, could be discontinued before ending rituximab treatment in all patients. Two patients were treated with rituximab only during recurrences and had sustained remissions.
Rituxan as been rapidly adopted and is now extensively used. It should be consideeds it appropriate in steroid refractory disease. There are no FDA approved therapies for this disease and Rituxan is off-label.
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