{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,17]],"date-time":"2026-10-17T15:52:08Z","timestamp":1792252328892,"version":"4.4.0"},"reference-count":46,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2025,10,9]],"date-time":"2025-10-09T00:00:00Z","timestamp":1759968000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"},{"start":{"date-parts":[[2025,10,9]],"date-time":"2025-10-09T00:00:00Z","timestamp":1759968000000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Acad Dermatol Venereol"],"published-print":{"date-parts":[[2026,3]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>\n                    Advances in genomics have redefined inflammatory linear verrucous epidermal naevus (ILVEN) as a mosaic inflammatory disorder with diverse molecular drivers, enabling more precise diagnostic and therapeutic strategies. This review aimed to summarize the published data on genotype\u2013phenotype correlations and associated targeted therapies in ILVEN and ILVEN\u2010like disorders. A structured literature search was conducted using PubMed, Embase and Google Scholar, focusing on peer\u2010reviewed studies describing mutations, pathomechanisms and treatment responses in ILVEN or ILVEN\u2010mimicking dermatoses. Search terms included \u201cILVEN\u201d, \u201cmosaic inflammatory dermatoses\u201d, \u201cgenetic mutations\u201d and \u201ctargeted therapy\u201d. Multiple genes have been implicated in ILVEN. Somatic mutations in\n                    <jats:italic>CARD14<\/jats:italic>\n                    activate NF\u2010\u03baB and IL\u201012\/23\/IL\u201017 signalling pathways and may be responsive to biologics such as secukinumab and ustekinumab. Mutations in\n                    <jats:italic>NSDHL<\/jats:italic>\n                    and\n                    <jats:italic>PMVK<\/jats:italic>\n                    , involved in cholesterol biosynthesis, are associated with ILVEN\u2010like presentations and may respond to topical statin\/cholesterol therapy.\n                    <jats:italic>KRT10<\/jats:italic>\n                    mutations, which affect keratinocyte differentiation, show favourable responses to crisaborole and acitretin.\n                    <jats:italic>GJA1<\/jats:italic>\n                    mutations disrupt connexin 43 function, resulting in ILVEN\u2010Erythrokeratodermia Variabilis et Progressiva (EKVP) overlap and may respond to retinoids.\n                    <jats:italic>HRAS<\/jats:italic>\n                    mosaicism activates the RAS\u2010MAPK pathway, supporting the theoretical use of MEK inhibitors. Recessive mosaicism in\n                    <jats:italic>ABCA12<\/jats:italic>\n                    can produce linear, ILVEN\u2010like lesions due to defective lipid barrier formation, with potential responsiveness to lipid\u2010replenishing therapies and IL\u201017 blockade. The molecular characterization of ILVEN and related disorders supports a shift toward precision dermatology. As accessibility increases, genotype\u2010directed therapy may replace empiric approaches with more targeted, effective and personalized management frameworks.\n                  <\/jats:p>","DOI":"10.1111\/jdv.70098","type":"journal-article","created":{"date-parts":[[2025,10,9]],"date-time":"2025-10-09T12:55:22Z","timestamp":1760014522000},"page":"353-360","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":6,"title":["The evolving genetic landscape of\n                    <scp>ILVEN<\/scp>\n                    : A comprehensive review"],"prefix":"10.1111","volume":"40","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-8088-4509","authenticated-orcid":false,"given":"Marra","family":"Aghajani","sequence":"first","affiliation":[{"name":"The Skin Hospital  Sydney New South Wales Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jessie Tong","family":"Lu","sequence":"additional","affiliation":[{"name":"The Skin Hospital  Sydney New South Wales Australia"},{"name":"School of Clinical Medicine University of New South Wales Medicine and Health  Sydney New South Wales Australia"},{"name":"Department of Dermatology Liverpool Hospital  Liverpool New South Wales Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"John W.","family":"Frew","sequence":"additional","affiliation":[{"name":"The Skin Hospital  Sydney New South Wales Australia"},{"name":"School of Clinical Medicine University of New South Wales Medicine and Health  Sydney New South Wales Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Deshan F.","family":"Sebaratnam","sequence":"additional","affiliation":[{"name":"The Skin Hospital  Sydney New South Wales Australia"},{"name":"School of Clinical Medicine University of New South Wales Medicine and Health  Sydney New South Wales Australia"},{"name":"Department of Dermatology Liverpool Hospital  Liverpool New South Wales 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