Abstract: Paraneoplastic pemphigus (PNP) is a rare, lifethreatening, autoimmune, and mucocutaneous blistering disease associated with neoplasia. The clinical features of PNP are mucosal erosion and polymorphic skin lesions in patients with concomitant tumor. Due to the complexity of the clinical manifestations, the misdiagnosis rate of the first diagnosis is high. In recent years, the pathogenesis and clinical manifestations of this disease have been more intensively studied. This article expounded and summarized PNP in terms of its pathogenesis, clinical manifestation, pathological and immunological features, as well as diagnosis and treatment to better understand this disease and improve the effect of diagnosis and treatment.

Key words: paraneoplastic pemphigus, mucosal erosion, diagnosis