Orphanet: Primary non essential cutis verticis gyrata

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Primary non-essential cutis verticis gyrata

Disease definition

Primary non-essential cutis verticis gyrata is a rare, genetic, dermis disorder characterized by slowly progressive thickening of the scalp, which becomes raised and forms ridges and furrows with symmetrical distribution resembling the cerebral gyri and cannot be flattened by traction or pressure, associated with ophthalmologic (e.g. congenital cataract) and/or neurological abnormalities (e.g. intellectual disability, epilepsy, microcephaly, encephalopathy).


Classification level: Disorder
  • Synonym(s): -
  • Prevalence: -
  • Inheritance: -
  • Age of onset: -
  • ICD-10: Q82.8
  • OMIM: 219300
  • UMLS: C4755295
  • MeSH: -
  • GARD: -
  • MedDRA: -
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