色素失禁症研究进展

摘要

色素失禁症（Incontinentia pigmenti，IP）是一种X连锁显性遗传性疾病，其主要症状表现为患儿出生时或生后两周皮肤出现红斑及水疱，继水疱后在相同部位出现疣状损害，之后表现为奇特的网状色素沉着。IP还伴有其他系统症状。本病大多愈后良好。本文就该病发病机制、症状及诊断、鉴别诊断作一综述。%Incontinentia pigmenti is an X-linked dominant genetic disease.Its main symptoms are the skin appears erythema and bullous when children born or two weeks after the birth,and having verrucous following in the same position. The last,these become a peculiar reticular pigment.IP is also accompanied other systems symptoms.Most patients of the prognosis is good.This is a review about the pathogenesis of the disease,symptoms and differential diagnosis.