ICD-11 classes
20 Developmental anomalies
Multiple developmental anomalies or syndromes
LD27 — Syndromes with skin or mucosal anomalies as a major feature
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 #16212