Clinical Characterization of Oral Symptoms in Paraneoplastic Pemphigus

Objectives: The purpose of this study was to analyze the characteristic feature of oral symptom of paraneoplastic pemphigus as PNP.
Methods: Seven PNP patients who had diagnosed as typical PNP were evaluated. We focused on the clinical findings regarding to oral mucosal symptoms. In addition, laboratory data including serologic, immunologic, histologic and immunohistologic results were also analyzed.
Results: The patient in this study consisted of 3 males and 4 females. The age at the first visit between 51 and 76 years old. (median age 62.0y). The majority of accompanying tumor was malignant lymphoma. Only one patient accompanied thymoma as solid tumor. As a clinical phenotype of PNP, relation between skin and oral mucosal symptoms were analyzed. At the initial phase of disease, 3 patients had oral symptom alone followed by the skin symptoms, 2 patients had both oral and skin symptom, the other patient had oral or skin symptom alone respectively. In all of patients, severe erosion, ulcer and lichenoid mucosal eruption was found in the oral mucosa. Remarkably, all of patient displayed severe cheilitis such as bloody crust and extensive erosion at the initial manifestations of PNP. In histologiy, both blister formaotion and interface dermatitis. In all of the patients, direct immunofluorescence was positive and circulating anti-Dsg3 autoantibody was detected by ELISA. In prognosis, 3 cases died within 6 months from the point of diagnosis.
Conclusions: Oral mucosal symptoms are frequently recognized as the initial symptom of autoimmune bullous diseases. In this study, oral symptoms of PNP was heterogenous compare to such as pemphigus vulgaris or mucous membrane pemphigoid. Our data indicated that heterogenous stomatitis including blister formation and lichenoid eruption were found in all patients. These findings provably correlate with the autoimmune mechanism of both sides of humoral and cell-mediated immunity. We also showed severe cheilitis was the typical initial symptom of PNP. This study indicated the importance of oral symptoms in diagnosis of PNP. PNP is still fetal and recalcitrant disease, therefore, all clinician should understand and share the characteristic features of PNP in order to make an accurate diagnosis.