Epidermal nevus syndromes: New insights into whorls and swirls.

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.

journal_name

Pediatr Dermatol

journal_title

Pediatric dermatology

authors

Asch S,Sugarman JL

doi

10.1111/pde.13273

subject

Has Abstract

pub_date

2018-01-01 00:00:00

pages

21-29

issue

1

eissn

0736-8046

issn

1525-1470

journal_volume

35

pub_type

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