Orphanet: Netherton syndrome

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Netherton syndrome

Disease definition

Netherton syndrome (NS) is a skin disorder characterized by congenital ichthyosiform erythroderma (CIE), a distinctive hair shaft defect (trichorrhexis invaginata; TI) and atopic manifestations.


  • Synonym(s):
    • Bamboo hair syndrome
    • Comèl-Netherton syndrome
    • NS
  • Prevalence: 1-9 / 1 000 000
  • Inheritance: Autosomal recessive 
  • Age of onset: Infancy, Neonatal
  • ICD-10: Q80.8
  • OMIM: 256500
  • UMLS: C0265962
  • MeSH: -
  • GARD: 7182
  • MedDRA: 10062909

Detailed information

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Additional information

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