A Case Report of Refractory Pemphigus Vulgaris Treated by Rituximab

Abstract

Treatment of pemphigus vulgaris (PV) can be a challenge. Although the standard therapy consists of a systemic corticosteroid, immunosuppressant drugs, and intravenous gammaglobulin, the possibility of severe side effects or fatal complications often prevents us from continuing these therapies. Herein, we describe a case of severe PV treated by rituximab administration. The patient was a 37-year-old female who first noticed an erosion on the oral mucosa in 2004. She was referred to our hospital, and was diagnosed as PV based on the clinical presentation, skin biopsy findings, and titers of anti-desmoglein antibody. Treatment with a systemic corticosteroid and immunosuppressant drugs briefly relieved her symptoms; however, her disease worsened in severity in 2007. Switching of the immunosuppressant drugs and plasmapheresis were not effective. Pulsed steroid therapy with high-dose methylprednisolone had a transient effect; however, soon after, the patient developed exacerbation of the systemic erosions. Administration of rituximab at a dosage of 375 mg/m² once weekly for four weeks produced rapid clinical remission. During the 4 years of follow-up, her anti-desmoglein antibody titer has gradually increased; however, the clinical symptoms remain mild. Our experience in this case suggests that rituximab may have other clinical benefits in addition to ablating antibodies.