Treatment of severe pemphigus vulgaris with rituximab

Abstract

Pemphigus vulgaris is an autoimmune skin disease mediated by autoantibodies directed against desmoglein-3 and desmoglein-1 located on keratinocytes. Rituximab, a monoclonal anti-CD20 antibody depleting B-cells, offers an effective treatment possibility for therapy-resistant pemphigus vulgaris. Here, we present a case of 20-year-old female who did not respond sufficiently to conventional treatment with prednisolone, azathioprine, mycophenolate mofetil and intravenous immunoglobulins but underwent almost complete remission after rituximab treatment. It was given as two injections of 500mg each at an interval of two weeks in ICU setting.