Linear Segmental Verrucous Epidermal Nevi with Oral Mucosa Involvement: A Rare Case Report

Epidermal nevi (EN) are benign cutaneous hamartomas following the lines of Blaschko, originating from postzygotic mutations in embryonic ectoderm. Keratinocytic EN, a subtype, commonly involves the trunk and extremities, with rare oral mucosal involvement. We present a 10-year-old female with linear...

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Main Authors: Spandana D. Krishnamurthy, Amrita Hongal, M. Madhu
Format: Article
Language:English
Published: Wolters Kluwer Medknow Publications 2025-01-01
Series:Indian Journal of Paediatric Dermatology
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Online Access:https://journals.lww.com/10.4103/ijpd.ijpd_142_24
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author Spandana D. Krishnamurthy
Amrita Hongal
M. Madhu
author_facet Spandana D. Krishnamurthy
Amrita Hongal
M. Madhu
author_sort Spandana D. Krishnamurthy
collection DOAJ
description Epidermal nevi (EN) are benign cutaneous hamartomas following the lines of Blaschko, originating from postzygotic mutations in embryonic ectoderm. Keratinocytic EN, a subtype, commonly involves the trunk and extremities, with rare oral mucosal involvement. We present a 10-year-old female with linear, hyperpigmented, verrucous plaques on the left face, following Blaschko’s lines, accompanied by ipsilateral tongue discoloration and lip hypertrophy. No systemic abnormalities were noted. Oral verrucous EN (VEN) is rare, with only 16 cases documented globally, typically unilateral and corresponding to cutaneous lesions. Diagnosis relies on histopathology, and management is challenging due to limited guidelines. Treatment is generally unnecessary for asymptomatic cases, but extensive lesions may require surgical or alternative interventions. This case highlights a unique presentation of unilateral keratinocytic EN with ipsilateral oral involvement, emphasizing the need for awareness of its clinical spectrum and consideration of follow-up for potential changes.
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2319-7269
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publisher Wolters Kluwer Medknow Publications
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spelling doaj-art-1faa39cfc27d4f67bf014cd114a35e412025-08-20T02:11:34ZengWolters Kluwer Medknow PublicationsIndian Journal of Paediatric Dermatology2319-72502319-72692025-01-01261414310.4103/ijpd.ijpd_142_24Linear Segmental Verrucous Epidermal Nevi with Oral Mucosa Involvement: A Rare Case ReportSpandana D. KrishnamurthyAmrita HongalM. MadhuEpidermal nevi (EN) are benign cutaneous hamartomas following the lines of Blaschko, originating from postzygotic mutations in embryonic ectoderm. Keratinocytic EN, a subtype, commonly involves the trunk and extremities, with rare oral mucosal involvement. We present a 10-year-old female with linear, hyperpigmented, verrucous plaques on the left face, following Blaschko’s lines, accompanied by ipsilateral tongue discoloration and lip hypertrophy. No systemic abnormalities were noted. Oral verrucous EN (VEN) is rare, with only 16 cases documented globally, typically unilateral and corresponding to cutaneous lesions. Diagnosis relies on histopathology, and management is challenging due to limited guidelines. Treatment is generally unnecessary for asymptomatic cases, but extensive lesions may require surgical or alternative interventions. This case highlights a unique presentation of unilateral keratinocytic EN with ipsilateral oral involvement, emphasizing the need for awareness of its clinical spectrum and consideration of follow-up for potential changes.https://journals.lww.com/10.4103/ijpd.ijpd_142_24blaschkoid distributionkeratinocytic nevusverrucous epidermal nevus
spellingShingle Spandana D. Krishnamurthy
Amrita Hongal
M. Madhu
Linear Segmental Verrucous Epidermal Nevi with Oral Mucosa Involvement: A Rare Case Report
Indian Journal of Paediatric Dermatology
blaschkoid distribution
keratinocytic nevus
verrucous epidermal nevus
title Linear Segmental Verrucous Epidermal Nevi with Oral Mucosa Involvement: A Rare Case Report
title_full Linear Segmental Verrucous Epidermal Nevi with Oral Mucosa Involvement: A Rare Case Report
title_fullStr Linear Segmental Verrucous Epidermal Nevi with Oral Mucosa Involvement: A Rare Case Report
title_full_unstemmed Linear Segmental Verrucous Epidermal Nevi with Oral Mucosa Involvement: A Rare Case Report
title_short Linear Segmental Verrucous Epidermal Nevi with Oral Mucosa Involvement: A Rare Case Report
title_sort linear segmental verrucous epidermal nevi with oral mucosa involvement a rare case report
topic blaschkoid distribution
keratinocytic nevus
verrucous epidermal nevus
url https://journals.lww.com/10.4103/ijpd.ijpd_142_24
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AT amritahongal linearsegmentalverrucousepidermalneviwithoralmucosainvolvementararecasereport
AT mmadhu linearsegmentalverrucousepidermalneviwithoralmucosainvolvementararecasereport