葡萄酒色斑增厚机制的研究进展

Research progress on the hypertrophy mechanism of port-wine stain

葡萄酒色斑(Port-wine stain,PWS)是一种常见的先天性毛细血管畸形疾病,病程发展到中晚期时常出现病灶的增生肥厚和结节化改变,PWS增厚和结节化改变后的治疗方法不同于平坦型PWS。因此,对PWS增生机制的研究显得尤为重要,将有助于提出这类患者的针对性治疗方案。本文对增厚PWS的组织学与细胞学表型、组织内皮细胞的常见分子表现,以及近期研究发现的可能导致生殖细胞系与体细胞系PWS发生发展的突变基因等方面进行综述。

Port-wine stain is a common congenital capillary malformation disease,which can gradually progress to the hypertrophic and nodular PWS in the middle and late stages.The treatment method for hypertrophic lesions is different from flat lesions.Thus,the exploration of the hypertrophy mechanism of PWS appears to be particularly significant and will help to propose targeted treatments for this type of patients.In this paper,the histological and cytological phenotypes,common molecular manifestations of endothelial cells in hypertrophic port-wine stain tissue,as well as the recent finding of mutated genes that may lead to the pathogenesis and development of PWS were all reviewed.