Familial systematized epidermal nevus syndrome

J. F. Meschia, E. Junkins, K. J. Hofman

Research output: Contribution to journalArticlepeer-review

10 Scopus citations


Epidermal nevi are typically congenital but rarely familial. We report on a family in which 3 relatives have systematized epidermal nevi. The propositus also has evidence of a hemangioma and a hemangioendothelioma. Peripheral blood and skin fibroblast karyotypes of the propositus did not show evidence of mosaicism. Epidermal nevi have been associated with nondermatologic pathology, involving the nervous, vascular, and skeletal systems in sporadic cases. This report demonstrates that nondermatologic pathology can be also be associated with systematized epidermal nevi in a familial setting. The apparent skipping of generations may be explained by autosomal dominant inheritance with decreased penetrance.

Original languageEnglish (US)
Pages (from-to)664-667
Number of pages4
JournalAmerican journal of medical genetics
Issue number5
StatePublished - 1992


  • Blaschko's lines
  • Schimmelpenning-Feuerstein-Mims syndrome
  • epidermal nevus
  • hemangioendothelioma
  • hemangioma
  • ichthyosis hystrix
  • nevus sebaceous of Jadassohn
  • nevus unius lateris

ASJC Scopus subject areas

  • Genetics(clinical)


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