Basic and Clinical Research on Paraneoplastic Pemphigus

副肿瘤性天疱疮(PNP)是一种自身免疫性大疱性皮肤病,常伴发肿瘤.临床表现为严重的黏膜糜烂和多形性皮肤损害.PNP的主要病理学特点为表皮基底细胞层上棘细胞松解性水疱及个别坏死的角质形成细胞.患者血清中可存在致病性的自身抗体,其产生与伴发的肿瘤有关.早期诊断及手术及时切除肿瘤是成功治疗的关键.Paraneoplastic pemphigus (PNP) is an autoimmune blistering skin disease associated with neoplasmas. Clinically, it is characterized by severe mucosal erosions and various cutaneous lesions. Suprabas-al acantholysis and cleft with scattered necrotic keratinocytes are the unique histopathological features of PNP. The pathogenic autoantibodies existed in PNP sera, and their production was correlated with the associated tumor. Early detection and resection of the tumor are essential for the treatment of the disease.国家自然科学基金中文核心期刊要目总览(PKU)中国科技核心期刊(ISTIC)中国科学引文数据库(CSCD)05654-6583