网状色素性疾病(RPD)是一组以色素沉着和/或色素沉着斑过度为特征的遗传性和获得性皮肤病。遗传的RPDs包括对称性色素沉着症(DSH),普氏色素沉着症(DUH),北村(RAK)的网状色素沉着,道林-德戈斯病(DDD),先天性角化障碍(DKC),Naegeli-Franceschetti-Jadassohn综合征(NFJS),色素性网状皮肤病(DPR),和X连锁网状色素紊乱症。尽管网状色素沉着是这种疾病的共同特征,色素沉着的分布在这些疾病中有所不同,可能有色素沉着以外的临床表现。DSH,DUH,和RAK主要在东亚种族中报道。DDD在白种人中更常见,尽管亚洲国家也有报道。其他RPD没有种族偏爱。本文综述了临床、组织学,和遗传RPDs的遗传变异。
Reticulate pigmentary disorders (RPDs) are a group of inherited and acquired skin conditions characterized by hyperpigmented and/or hypopigmented macules. Inherited RPDs include dyschromatosis symmetrica hereditaria (DSH), dyschromatosis universalis hereditaria (DUH), reticulate acropigmentation of Kitamura (RAK), Dowling-Degos disease (DDD), dyskeratosis congenita (DKC), Naegeli-Franceschetti-Jadassohn syndrome (NFJS), dermatopathia pigmentosa reticularis (DPR), and X-linked reticulate pigmentary disorder. Although reticulate pattern of pigmentation is a common characteristic of this spectrum of disorders, the distribution of pigmentation varies among these disorders, and there may be clinical manifestations beyond pigmentation. DSH, DUH, and RAK are mostly reported in East Asian ethnicities. DDD is more common in Caucasians, although it is also reported in Asian countries. Other RPDs show no racial predilection. This article reviews the clinical, histological, and genetic variations of inherited RPDs.