We present a case of a 49-year-old Japanese woman with recalcitrant pemphigus vulgaris associated with multiple severe infections and very high anti-desmoglein 1 and 3 antibody titers. The lesions covered more than 70% of her body surface area. A combination of repeated steroid pulse therapy, plasmapheresis, and IVIG could not control the disease. However, a single cycle of rituximab therapy led to complete remission for 18 months, during her steroid dose was tapered. No adverse effects have been observed till date. Rituximab is a chimeric murine?human monoclonal antibody that targets the CD20 antigen found on B cells andcauses rapid depletion of this cell population. It has been approved for the treatment of non-Hodgkin B-cell lymphoma and rheumatoid arthritis in the United States. In addition, rituximab has been shown to be effective for the treatment of other autoimmune diseases such as systemic lupus erythematosus and pemphigus. This treatment should be considered for recalcitrant cases with persistent high titers of autoimmune antibodies despite combination therapy with corticosteroids, immunosuppressive agents, plasmapheresis, and IVIG. It can also be considered for cases of recurrence due to tapering of steroids and immunosuppressive agents. However, the adverse effects of immunosuppression by rituximab should be carefully considered for each individual patient.
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