鲜红斑痣相关综合征的诊疗进展  

Diagnosis and treatment of port-wine stains-associated syndromes

作　　者：刘青锋 刘莲 刁萍 李晓雪[1] 张婷[1] 陈浩天 刘绪 蒋献[1] Liu Qingfeng;Liu Lian;Diao Ping;Li Xiaoxue;Zhang Ting;Chen Haotian;Liu Xu;Jiang Xian(Department of Dermatology and Venereology,West China Hospital,Sichuan University,Laboratory of Dermatology,Clinical Institute of Inflammation and Immunology,Frontiers Science Center for Disease-related Molecular Network,West China Hospital,Sichuan University,Chengdu 610041,China)

机构地区：[1]四川大学华西医院皮肤性病科、四川大学疾病分子网络前沿科学中心免疫炎症研究院皮肤病学研究室,成都610041

出　　处：《中华皮肤科杂志》2024年第7期656-660,共5页Chinese Journal of Dermatology

基　　金：国家自然科学基金(82003373);四川大学华西医院学科卓越发展1.3.5工程项目(ZYJC21036)。

摘　　要：鲜红斑痣(PWS)是皮肤科常见的先天性血管疾病,表现为出生时或出生后不久于皮肤或黏膜出现粉红色或红色不规则斑片,常不高出皮肤表面。少部分PWS患者不仅表现为皮肤的血管畸形,还可累及眼睛、颅脑、四肢及内脏的血管,患者可出现青光眼、癫痫、四肢疼痛等表现,这一类疾病统称为PWS相关综合征。PWS相关综合征较为罕见,可累及多个系统,临床表现复杂,诊疗具有一定的难度。本文综述常见PWS相关综合征的临床表现、诊断、发病机制及治疗。Port-wine stains(PWS)are one of the common congenital vascular malformations in dermatology,clinically manifesting as pink or red irregular patches occurring on the skin or mucosa at birth or shortly thereafter,which are often not elevated above the skin surface.In a minority of patients,vascular malformations not only affect the skin,but also involve the eyes,brain,limbs and viscera.These patients are at risk for glaucoma,epilepsy,limb pain,and other clinical conditions.In general,these conditions are referred to as PWS-associated syndromes.These syndromes are rare diseases,can affect multiple systems and exhibit a variety of clinical manifestations,which pose challenges in their diagnosis and treatment.This review focuses on the clinical manifestations,diagnoses,pathogenesis and treatment of PWS-associated syndromes.

关 键 词：葡萄酒色痣 综合征 STURGE-WEBER综合征 PROTEUS综合征 神经皮肤综合征 血管畸形 淋巴管畸形 诊断 

分 类 号：R758.61[医药卫生—皮肤病学与性病学]