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Therefore, some authors suggest the concept of mosaic RASopathies to group these different disorders.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>In this paper, we describe three new cases of syndromic epidermal nevi caused by mosaic<jats:italic>HRAS<\/jats:italic>variants: one associating an extensive keratinocytic epidermal nevus with hypomastia, another with extensive mucosal involvement and a third combining a small sebaceous nevus with seizures and intellectual deficiency. Moreover, we performed extensive literature of all cases of syndromic epidermal nevi and related disorders with confirmed pathogenic postzygotic variants in<jats:italic>HRAS, KRAS, NRAS<\/jats:italic>or<jats:italic>BRAF<\/jats:italic>.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Most patients presented with bone, ophthalmological or neurological anomalies. Rhabdomyosarcoma, urothelial cell carcinoma and pubertas praecox are also repeatedly reported.<jats:italic>KRAS<\/jats:italic>pathogenic variants are involved in 50% of the cases, especially in sebaceous nevi, oculoectodermal syndrome and encephalocraniocutaneous lipomatosis. They are frequently associated with eye and brain anomalies. Pathogenic variants in<jats:italic>HRAS<\/jats:italic>are rather present in syndromic keratinocytic epidermal nevi and phacomatosis pigmentokeratotica.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>This review delineates genotype\/phenotype correlations of syndromic epidermal nevi with somatic<jats:italic>RAS<\/jats:italic>and<jats:italic>BRAF<\/jats:italic>pathogenic variants and may help improve their follow-up.<\/jats:p><\/jats:sec>","DOI":"10.1136\/jmg-2023-109306","type":"journal-article","created":{"date-parts":[[2024,1,30]],"date-time":"2024-01-30T18:21:32Z","timestamp":1706638892000},"page":"jmg-2023-109306","update-policy":"https:\/\/doi.org\/10.1136\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":4,"title":["Mosaic RASopathies concept: different skin lesions, same systemic 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