Paraneoplastic pemphigus: A case of long-term survival associated with systemic lupus erythematosus and polymyositis

A 35-year-old man presented with a generalized bullous eruption and oral ul ceration. He bad been diagnosed as having systemic lupus erythematosus and pelvic Castleman disease (hyaline-vascular type) in the past. Histologic, d irect and indirect immunofluorescence and immunoprecipitation studies confi rmed a diagnosis of paraneoplastic pemphigus (PNP), Initially several medic al treatments were tried unsuccessfully. The pelvic tumor was surgically re moved and the mucocutaneous lesions slowly regressed. Four years after pres entation, he developed polymyositis which was completely controlled with sh ort courses of corticosteroids, There was no evidence of relapse of PNP or lupus erythematosus at that time. At the 6-year follow-up he showed no clin ical evidence of PNP, lupus erythematosus or polymyositis without requiring immunosuppressive therapy. This case emphasizes the fact that patients wit h benign-neoplasm-associated PNP may undergo complete remission of the auto immune disease upon complete excision of the tumor. This case also stresses the possible association of PNP with other autoimmune diseases such as lup us erythematosus and polymyositis.