Epidermal nevus syndromes: New insights into whorls and swirls. Issue 1 (16th October 2017)

• Record Type: Journal Article
• Authors: Asch, Sarah; Sugarman, Jeffrey L.
• Is Part Of: Pediatric dermatology. Volume 35:Issue 1(2018)
• Page Start: 21
• Page End: 29
• Publication Date: 2017-10-16
• Subjects: Becker's nevus -- congenital hemidysplasia with ichthyosiform nevus and limb defects syndrome -- cutaneous‐skeletal hypophosphatemia syndrome -- epidermal nevus -- inflammatory linear verrucous epidermal nevus -- nevus comedonicus -- nevus sebaceous -- phakomatosis pigmentokeratotica -- porokeratotic adnexal ostial nevus; Pediatric dermatology -- Periodicals; Children -- Diseases -- Periodicals; 618.925
• DOI: 10.1111/pde.13273
• Languages: English
• ISSNs: 0736-8046

Abstract

Abstract: Knowledge of the molecular underpinnings of many epidermal nevi and epidermal nevus syndrome has expanded rapidly in recent years. In this review and update on epidermal nevus syndrome, we will cover recent genetic discoveries involving epidermal nevi, including nevus sebaceus, keratinocytic epidermal nevus, nevus comedonicus, congenital hemidysplasia with ichthyosiform nevus and limb defects syndrome, phakomatosis pigmentokeratotica, Becker's nevus, porokeratotic adnexal ostial nevus, inflammatory linear verrucous epidermal nevi, and cutaneous‐skeletal hypophosphatemia syndrome. We will discuss how newly defined mutations relate to the biology reflected in the cutaneous patterns seen in these mosaic disorders and how new molecular data has informed our understanding of these diseases and shaped management decisions.