Retrospective Analysis of Seven Patients with Paraneoplastic Pemphigus

Objective To determine the clinical findings, treatment and clinical outcomes of paraneoplastic pemphigus(PNP),arare,life-threatening,autoimmuneblisteringdiseaseassociatedwithneoplasia.Methods Seven patients with PNP that were diagnosed between 2008 and 2013 were retrospectively analyzed. Analysis of the clinical features, clinical outcomes, underlying neoplasia, histological features and laboratory fingdings were performed. Results All of the patients had severe mucosal involvement. Erythema multiforme, lichen planus-like eruptions and bullous lesions were commonly observed skin rashes. The most common histological features were interface dermatitis and apoptotic keratinocytes. CT scan appeared solitary mass in these patient, with Castleman's disease(n=6)as the most frequent. Seven patients were followed for 5~60 months. Three patients died dut to postoperation infection(n =1), serious infections in multiple sites before operation(n =1) and drug discontinuance postoperation(n=1). Conclusion Castleman's disease is the most frequent neoplasm in these patients. Early diagnosis and prompt removal of the tumor are key to the management of this disease, the appropriate treatment of postoperation can improve the prognosis.