进行性对称性红斑角化症（PSEK）包含一组临床及遗传学异质性较强的疾病，既往研究认为n GJB3和n GJB4是其主要致病基因。随着遗传学研究快速进展，国内外团队近年陆续发现PSEK的全新致病基因n GJA1、n KDSR、n KRT83、n TRPM4，促使PSEK的临床特征和遗传学发病机制得到进一步认识。值得注意的是，我国皮肤科医生既往普遍将长岛型掌跖角化症误诊为PSEK，随着长岛型掌跖角化症致病基因被发现，两种疾病的区别应逐步得到认识。n “,”Progressive symmetric erythrokeratodermia (PSEK) comprises a group of clinically and genetically heterogeneous diseases. Previous research have identified n GJB3 and n GJB4 as the leading genetic causes of this disorder. With the rapid development of genetics, n GJA1, n KDSR, n KRT83 and n TRPM4 have been identified as the new causative genes for PSEK, leading to a further understanding of its clinical features and genetic mechanisms. It′s worth noting that Nagashima-type palmoplantar keratosis was often misdiagnosed as PSEK by our domestic dermatologists. Due to the identification of n SERPINB7 as the causative gene of Nagashima-type palmoplantar keratosis recently, differentiation between the two disorders could be easily distinguished.n