LD27.2

ICD-11 MMS 2026-01

Syndromic ichthyosis

Description

Hereditary disorders in which ichthyosis is associated with significant other abnormalities.

Index terms

  • Syndromic ichthyosis
  • X-linked ichthyosis syndromes
  • Ichthyosis follicularis – atrichia – photophobia
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  • IFAP - [Ichthyosis follicularis – atrichia – photophobia] syndrome
  • ichthyosis follicularis - alopecia - photophobia
  • Autosomal ichthyosis syndromes with hair abnormalities
  • Netherton syndrome
  • Comèl-Netherton syndrome
  • Netherton disease
  • Trichorrhexis invaginata
  • Bamboo hair
  • Ichthyosis – hypotrichosis syndrome
  • PIBIDS syndrome
  • Trichothiodystrophy - sun sensitivity
  • Trichothiodystrophy type F
  • SIBIDS syndrome
  • Trichothiodystrophy - osteosclerosis
  • Autosomal ichthyosis syndromes with neurological manifestations
  • Autosomal ichthyoses with fatal disease course
  • Cerebral dysgenesis – neuropathy – ichthyosis – keratoderma syndrome
  • CEDNIK - [Cerebral dysgenesis – neuropathy – ichthyosis – keratoderma syndrome] syndrome
  • Other autosomal ichthyosis syndromes
  • Ichthyosis - oral and digital anomalies
  • Clayton Smith-Donnai syndrome
  • Keratitis – ichthyosis – deafness syndrome
  • Hystrix-like ichthyosis - deafness
  • KID - [Keratitis – ichthyosis – deafness syndrome] syndrome
  • KID/HID - [Keratitis - ichthyosis - deafness/Hystrix-like ichthyosis - deafness] syndrome
  • Keratitis - ichthyosis - deafness/Hystrix-like ichthyosis - deafness
  • Senter syndrome
  • Ichthyosis – prematurity syndrome
  • Ichthyosis as component of other specified genetic syndrome

Exclusions from above levels

  • http://id.who.int/icd/entity/733825440 – Inborn errors of metabolism

Foundation children elsewhere

  • 5C52.03 – Sjögren-Larsson syndrome

Sibling codes

  • LD27.0 – Ectodermal dysplasia syndromes
  • LD27.1 – Xeroderma pigmentosum
  • LD27.3 – Genetic syndromes with hypertrichosis
  • LD27.4 – Genetic syndromes affecting nails
  • LD27.5 – Genetic hamartoneoplastic syndromes affecting the skin
  • LD27.6 – Genetic lipodystrophy
  • LD27.Y – Other specified syndromes with skin or mucosal anomalies as a major feature
  • LD27.Z – Syndromes with skin or mucosal anomalies as a major feature, unspecified

Foundation entity

Syndromic ichthyosis – http://id.who.int/icd/entity/488102959

Part of: LD27: Syndromes with skin or mucosal anomalies as a major feature › 20: Developmental anomalies