Paraneoplastic pemphigus: a review

Abstract

Paraneoplastic pemphigus (PNP) is a relatively recently described autoimmune disorder, distinguished from pemphigus vulgaris or foliaceus by specific clinical, histologic and immunologic criteria. In the present review, 18 cases of paraneoplastic pemphigus reported so far are discussed. The polymorphism, extent and persistence of skin and mucosal lesions are discussed while the problems of differential diagnosis and the significance of the associated neoplasms are also stressed. The underlying malignancy in the majority of the cases has been of lymphoid origin and has preceded the clinical presentation of pemphigus. All patients have had oral and cutaneous lesions. In 66.6% of the patients there was a poor response to therapy.