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Epidermal Nevus Syndromes

Abstract
The term epidermal nevus syndrome (ENS) was originally used as an all-encompassing term used to describe epidermal nevi in association with other syndromic features. In more recent years, this term was expanded to include several more well-defined neurocutaneous syndromes and their association with an epidermal nevus. The genetic basis of many of these syndromes has been elucidated in recent years. However, much is still to be learned about the genotype-phenotype correlation. Common epidermal nevi include non-epidermolytic keratinocyte nevus, nevus sebaceous, nevus comedonicus, Becker’s nevus, and CHILD nevus. These are hamartomas of the skin, which are grouped as either organoid or keratinocytic.[1] The syndromes included within this umbrella term are somewhat controversial. Therefore, the most frequently included syndromes will be discussed further. Schimmelpenning syndrome, phacomatosis pigmentokeratotica, nevus comedonicus syndrome, and Becker’s nevus syndrome are among the syndromes associated with organoid epidermal nevi. The syndromes associated with keratinocytic nevi include Proteus syndrome and congenital hemidysplasia with ichthyosiform nevus and limb defects (CHILD) syndrome.[2]

Publisher

StatPearls Publishing
Treasure Island (FL)

Language

eng

PubMed ID

32644429

Citation

Nicholson CL, Daveluy S: Epidermal Nevus Syndromes. StatPearls. StatPearls Publishing, 2020, Treasure Island (FL).
Nicholson CL, Daveluy S. Epidermal Nevus Syndromes. StatPearls. StatPearls Publishing; 2020.
Nicholson CL & Daveluy S. (2020). Epidermal Nevus Syndromes. In StatPearls. Treasure Island (FL): StatPearls Publishing
Nicholson CL, Daveluy S. Epidermal Nevus Syndromes. StatPearls. Treasure Island (FL): StatPearls Publishing; 2020.
* Article titles in AMA citation format should be in sentence-case
TY - CHAP T1 - Epidermal Nevus Syndromes BT - StatPearls A1 - Nicholson,Cynthia L., AU - Daveluy,Steven, Y1 - 2020/01// PY - 2020/7/10/pubmed PY - 2020/7/10/medline PY - 2020/7/10/entrez N2 - The term epidermal nevus syndrome (ENS) was originally used as an all-encompassing term used to describe epidermal nevi in association with other syndromic features. In more recent years, this term was expanded to include several more well-defined neurocutaneous syndromes and their association with an epidermal nevus. The genetic basis of many of these syndromes has been elucidated in recent years. However, much is still to be learned about the genotype-phenotype correlation. Common epidermal nevi include non-epidermolytic keratinocyte nevus, nevus sebaceous, nevus comedonicus, Becker’s nevus, and CHILD nevus. These are hamartomas of the skin, which are grouped as either organoid or keratinocytic.[1] The syndromes included within this umbrella term are somewhat controversial. Therefore, the most frequently included syndromes will be discussed further. Schimmelpenning syndrome, phacomatosis pigmentokeratotica, nevus comedonicus syndrome, and Becker’s nevus syndrome are among the syndromes associated with organoid epidermal nevi. The syndromes associated with keratinocytic nevi include Proteus syndrome and congenital hemidysplasia with ichthyosiform nevus and limb defects (CHILD) syndrome.[2] PB - StatPearls Publishing CY - Treasure Island (FL) UR - https://www.unboundmedicine.com/medline/citation/32644429/StatPearls:_Epidermal_Nevus_Syndromes L2 - https://www.ncbi.nlm.nih.gov/books/NBK559003 DB - PRIME DP - Unbound Medicine ER -
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