ICD-11 classes
05 Endocrine, nutritional or metabolic diseases
Metabolic disorders
Inborn errors of metabolism
5C50 — Inborn errors of amino acid or other organic acid metabolism
5C50.2 — Disorders of histidine metabolism
5C50.21 — Urocanic aciduria
ICD-11 5C50.21 — Urocanic aciduria
This is an autosomal recessive metabolic disorder caused by a deficiency of the enzyme urocanase. It is a secondary disorder of histidine metabolism.
The diagnosis includes nothing.
The diagnosis excludes nothing.
It has no clarifying diagnoses.