Clinical Characterization of Oral Symptoms in 6 Paraneoplastic Pemphigus Patients

Abstract

Paraneoplastic pemphigus（PNP）is a fetal autoimmune blistering disease mainly accompanied by a hematologic tumor manifesting various severe mucocutaneous lesions. To clarify the oral symptoms of PNP, we clinically analyzed six cases of PNP. The accompanying tumor was follicular lymphoma or thymoma. As typical oral symptoms, severe erosion, ulcer and lichenoid mucosal eruption in the oral mucosa and crust, blood crust and erosion of the lip were confirmed as the first manifestations of PNP. In addition, histopathologically, suprabasilar acantholysis and interface dermatitis caused by inflammatory cell infiltration were found. These clinical and histopathological characteristics were correlated with the autoimmune mechanism of both sides due to humoral and cell-mediated immunity. Given that oral symptoms are the first to occur, PNP patients are likely to visit a dentist for relief; as such, dentists should understand the pathophysiology, clinical manifestations and histopathological findings of PNP in order to make an accurate diagnosis in their dental practice.