EC20.01

ICD-11 MMS 2026-01

X-linked ichthyosis

Description

X-linked ichthyosis is an X-linked recessive genodermatosis associated with steroid sulfatase deficiency and elevated plasma cholesterol sulfate. Generalised scaling is present at or shortly after birth, most prominently over the extremities, neck, trunk, and buttocks. It occurs only in males and may be associated with testicular disease and corneal opacities.

Index terms

  • X-linked ichthyosis
  • Sex-linked ichthyosis
  • X-linked ichthyosis with steryl-sulfatase deficiency
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  • X-linked recessive ichthyosis

Sibling codes

  • EC20.00 – Ichthyosis vulgaris
  • EC20.02 – Autosomal recessive congenital ichthyosis
  • EC20.03 – Keratinopathic ichthyoses
  • EC20.0Y – Other specified or unclassifiable non-syndromic ichthyosis

Foundation entity

X-linked ichthyosis – http://id.who.int/icd/entity/1466487054

Part of: EC20.0: Non-syndromic ichthyosis › EC20: Genetic disorders of keratinisation › 14: Diseases of the skin