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Linear nevus sebaceus syndrome

Disease definition

Linear nevus sebaceous syndrome (LNSS) is characterized by the association of a large sebaceous nevus, usually appearing on the face or on the scalp, with a broad spectrum of abnormalities that may affect every organ system, including the central nervous system (brain neoplasms, hemimegalencephaly and lateral ventricle enlargement).


Classification level: Disorder
  • Synonym(s):
    • Nevus sebaceus of Jadassohn
    • Nevus sebaceus syndrome
    • Organoid nevus syndrome
    • Schimmelpenning syndrome
    • Solomon syndrome
  • Prevalence: Unknown
  • Inheritance: Not applicable 
  • Age of onset: Childhood
  • ICD-10: Q85.8
  • OMIM: 163200
  • UMLS: C0265329  C3854181
  • MeSH: -
  • GARD: 10291
  • MedDRA: -
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