Paraneoplastic pemphigus

Abstract

Paraneoplastic pemphigus has been established as a newly recognized but distinctive syndrome. The syndrome has distinctive clinical, histologic, and immunopathologic features that are consistent between affected individuals. It is intriguing that the autoimmune disease is associated with a very small number of unusual lymphoreticular malignancies. Its very restricted association is encouraging in that it implies there may be a common and identifiable mechanism by which these tumors induce the autoimmunity. Future important goals include the following. First, the genes that encode the as yet uncharacterized protein antigens must be identified and their exact nature determined. It is possible that one of these antigens may represent a biologically important epithelial adhesion molecule. Second, the mechanism or mechanisms by which the tumor cells drive the autoimmune disease must be determined. This may have broader implications for other paraneoplastic diseases such as tumor-associated autoimmune cytopenias or myasthenia. Finally, defining the basic mechanisms of disease induction may lead to improved treatment for the disease. At present it remains a devestating disease with a frighteningly high mortality. Fortunately, recent early diagnosis and intervention with combined prednisone-cyclosporine therapy have provided at least some hope of controlling the disease.