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
EC20.00 — Ichthyosis vulgaris

ICD-11 EC20.00 — Ichthyosis vulgaris

Ichthyosis vulgaris accounts for 95% of all cases of hereditary ichthyosis. It is an autosomal dominant condition due to filaggrin gene mutations. At birth the skin may appear normal but it gradually becomes dry, rough and scaly, with most signs and symptoms appearing by the age of 5. Ichthyosis vulgaris can affect all parts of the skin surface including the face and scalp though the limb flexures are usually spared. Hyperlinearity of the palms is a characteristic feature. It is closely associated with the development of atopic eczema.

The diagnosis includes nothing.

The diagnosis excludes nothing.

It has no clarifying diagnoses.

The diagnosis is coded elsewhere:

• Non-syndromic ichthyosis #15952
• Non-syndromic ichthyosis #16212