ICD-11 classes
14 Diseases of the skin
Skin disorders involving specific cutaneous structures
Disorders of the epidermis and epidermal appendages
Disorders of epidermal keratinisation
ED50 — Ichthyoses
EC20.0 — Non-syndromic ichthyosis
EC20.01 — X-linked ichthyosis
ICD-11 EC20.01 — X-linked ichthyosis
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.
The diagnosis includes nothing.
The diagnosis excludes nothing.
It has no clarifying diagnoses.
The diagnosis is coded elsewhere:
- Inborn errors of sterol metabolism #4819
- Hereditary corneal dystrophies #9419
- Non-syndromic ichthyosis #15952
- Non-syndromic ichthyosis #23355