Epidermal nevus syndromes.

Abstract:

:The term "epidermal nevus syndrome" (ENS) has been used to describe the association of epidermal hamartomas and extra-cutaneous abnormalities. Epidermal nevi follow the lines of Blaschko. The majority of the extra-cutaneous manifestations involve the brain, eye, and skeletal systems. Several subsets with characteristic features have been delineated including the nevus sebaceous syndrome, Proteus syndrome, CHILD syndrome, Becker nevus syndrome, nevus comedonicus syndrome, and phakomatosis pigmentokeratotica. Epidermal nevi have been associated with benign and malignant neoplasms. Advances in molecular biology have revealed that the manifestations of ENS are due to genomic mosaicism. It is likely that the varied clinical manifestations of ENS are due in great part to the functional effects of specific genetic defects. Optimal management of the patient with ENS involves an interdisciplinary approach. Amelioration of the cutaneous features of ENS has been difficult but there have been advances, especially in the use of lasers.

journal_name

Semin Cutan Med Surg

authors

Sugarman JL

doi

10.1016/j.sder.2008.03.006

subject

Has Abstract

pub_date

2007-12-01 00:00:00

pages

221-30

issue

4

eissn

1085-5629

issn

1558-0768

pii

S1085-5629(08)00025-4

journal_volume

26

pub_type

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