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Delayed response of oral pemphigus vulgaris to rituximab treatment


European Journal of Dermatology. Volume 16, Number 3, 266-70, May-June 2006, Therapy

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Author(s) : Andrea Niedermeier, Petra Wörl, Sandra Barth, Gerold Schuler, Michael Hertl

Summary : Rituximab is a monoclonal antibody directed against the CD20 antigen of B cells, which has been developed for the treatment of lymphomas and has been successfully used in the treatment of recalcitrant pemphigus vulgaris. Here, we report on a 26-year-old patient with an 18 month history of pemphigus vulgaris of the oral mucosa where treatment with rituximab led to a delayed but sustained therapeutic response. This case is of interest since most of the previous reports have described a more rapid clinical improvement of refractory pemphigus by rituximab treatment. The delayed clinical response to rituximab of the present case may be explained by the persistence of long-lived plasma cells that continued to produce pathogenic autoantibodies against desmoglein 3.

Keywords : IVIG, intravenous immunoglobulins, PF, pemphigus foliaceus, PV, pemphigus vulgaris

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