ArticlePediatric dermatology2022
Inflammatory linear verrucous epidermal nevus (ILVEN) encompasses a spectrum of inflammatory mosaic disorders.
Article in Pediatric dermatology, 2022. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 15 papers.
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Who cites it
15 citing papers in PubMed, 33 citations in OpenAlex.
- [Incontinentia pigmenti-the skin never forgets].Dermatologie (Heidelberg, Germany) · 2026Article
- Eruptive pruritic papular porokeratosis responsive to JAK1 inhibition.JAAD case reports · 2026Article
- Cutis Marmorata Telangiectatica Congenita: An Atypical Clinical Presentation of Vascular Anomalies.Acta dermato-venereologica · 2026Article
- Successful Treatment of Linear Porokeratosis with Ivarmacitinib in an Adolescent: A Case Report.Clinical, cosmetic and investigational dermatology · 2026Article
- Unmasking Linear Psoriasis: A Case of ILVEN-Like Presentation and Topical Treatment Success.Clinical case reports · 2025Article
- Psoriasis in the Context of Dermatologic Disorders: A Comprehensive Overview.Diseases (Basel, Switzerland) · 2025Review
- Linear epidermal naevus associated with a novel mosaic heterozygous PTPN11 variant.The British journal of dermatology · 2025Article
- Darier disease: Current insights and challenges in pathogenesis and management.Journal of the European Academy of Dermatology and Venereology : JEADV · 2025Review
- Inflammatory linear verrucous epidermal nevus successfully treated with ustekinumab.JAAD case reports · 2025Article
- Linear erythematous papulovesicular rash in a newborn.JAAD case reports · 2025Article
- Lichen Striatus: An Updated Review.Current pediatric reviews · 2025Review
- Ustekinumab is effective in the treatment of linear psoriasis: a case report and literature review.Frontiers in immunology · 2025Review
- Nail Lichen Striatus and Its Differential Diagnoses.Skin appendage disorders · 2024Review
- Adult-onset inflammatory linear verrucous epidermal nevus successfully treated with intralesional steroid.JAAD case reports · 2024Article
- A blaschkoid birthmark.JAAD case reports · 2024Article
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Authors and funding
7 authors at 5 institutions in 2 countries.
Funding
Abstract
backgroundInflammatory linear verrucous epidermal nevus (ILVEN) is a rare skin disease characterized by pruritic erythematous scaly plaques distributed along the lines of Blaschko. Two cases of ILVEN with CARD14 mutations and one case with a GJA1 mutation have been previously reported.
objectiveTo elucidate the genetic cause of a cohort of patients diagnosed based on clinical and histopathological evaluation with ILVEN.
methodsWe recruited patients diagnosed with ILVEN based on clinical and histopathological criteria. Exome sequencing of affected skin with or without blood/saliva was performed and germline and somatic pathogenic variants were identified.
resultsFive patients were enrolled. All had skin lesions from birth or early childhood. Two patients developed psoriasis vulgaris after the diagnosis of ILVEN. The first had a germline heterozygous CARD14 mutation and a post-zygotic hotspot mutation in KRT10. The histopathologic evaluation did not show epidermolytic hyperkeratosis. The second had a post-zygotic hotspot mutation in HRAS. Her ILVEN became itchy once psoriasis developed. One patient was re-diagnosed with linear porokeratosis based on a germline mutation in PMVK and a post-zygotic second-hit mutation. Two patients were re-diagnosed with congenital hemidysplasia with ichthyosiform nevus and limb defect nevus based on germline NSDHL mutations.
conclusionILVEN is a clinical descriptor for a heterogenous group of mosaic inflammatory disorders. Genetic analysis has the potential to more precisely categorize ILVEN and permits pathogenesis-directed therapies in some cases.
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