Using Rituximab (Anti-CD20 Antibody) in a Patient with Paraneoplastic Pemphigus
October 2003 | Volume 2 | Issue 5 | Case Reports | 564 | Copyright © October 2003
Monica B. Schadlow, MD; Grant J. Anhalt, MD and Animesh A. Sinha, Md, PhD
Abstract
Paraneoplastic pemphigus (PNP) is a rare autoimmune mucocutaneous blistering disease that is commonly
associated with underlying B-cell neoplasms. There is no standard therapy for PNP. Potent immunosuppression
has been the only potentially effective treatment in the setting of malignancy because there is no correlation
between tumor burden and activity of disease. Two recent case reports have noted the resolution of
lesions of PNP after treatment of the underlying CD20+ B-cell lymphomas with rituximab. Rituximab is an
anti-CD20 antibody that has had some success in treating proliferative B-cell disorders. We report a case of
PNP in the setting of B-cell lymphoma that did not respond to this novel therapy, and discuss rituximab’s putative
mechanism of action along with the clinical settings in which this novel therapy may prove useful in the
treatment of PNP. 
