A Germline Missense Variant in the NSDHL Gene Underlies Inflammatory Linear Verrucous Epidermal Nevus

Inflammatory linear verrucous epidermal nevus (ILVEN) is a rare skin disorder characterized by pruritic, erythematous, and scaly plaques following Blaschko's lines. Known genetic causes include somatic variants in CARD14 and GJA1. In addition, a similar phenotype of congenital hemidysplasia with ichthyosiform nevus and limb defects (CHILD) nevus has been associated with germline pathogenic variants in NSDHL encoding a NAD(P)-dependent steroid dehydrogenase-like protein essential for cholesterol biosynthesis. Here we report a case of ILVEN caused by a previously unreported germline pathogenic variant in NSDHL.

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Publication Details

Journal
Pediatric Dermatology
Published
2026-09-11
DOI
https://doi.org/10.1111/pde.70355
Primary Topic
Genetic and rare skin diseases.
Type
article
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article

A Germline Missense Variant in the NSDHL Gene Underlies Inflammatory Linear Verrucous Epidermal Nevus

Janan Mohamad, Ofer Sarig, Eli Sprecher, Kiril Malovitski et al.
Pediatric Dermatology
Genetic and rare skin diseases.
article

A Germline Missense Variant in the NSDHL Gene Underlies Inflammatory Linear Verrucous Epidermal Nevus

Janan Mohamad, Ofer Sarig, Eli Sprecher, Kiril Malovitski, Jacob Mashiah, Yarden Feller, Moran Furman, Liat Samuelov, Moshe Giladi, Eylon Sharoni, Nabih Elias
article en

Abstract

Inflammatory linear verrucous epidermal nevus (ILVEN) is a rare skin disorder characterized by pruritic, erythematous, and scaly plaques following Blaschko's lines. Known genetic causes include somatic variants in CARD14 and GJA1. In addition, a similar phenotype of congenital hemidysplasia with ichthyosiform nevus and limb defects (CHILD) nevus has been associated with germline pathogenic variants in NSDHL encoding a NAD(P)-dependent steroid dehydrogenase-like protein essential for cholesterol biosynthesis. Here we report a case of ILVEN caused by a previously unreported germline pathogenic variant in NSDHL.

Pediatric Dermatology
Tel Aviv University (IL), Tel Aviv Sourasky Medical Center (IL)
Good health and well-being
Openalex Percentile: Top 11%
Genetic and rare skin diseases.
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A Germline Missense Variant in the NSDHL Gene Underlies Inflammatory Linear Verrucous Epidermal Nevus — Janan Mohamad, Ofer Sarig, et al. · Pediatric Dermatology (2026) | TGRS Research Map | TGRS