ICD-11 classes
14 Diseases of the skin
Genetic and developmental disorders affecting the skin
5C56.30 — Mucopolysaccharidosis type 1

ICD-11 5C56.30 — Mucopolysaccharidosis type 1

Mucopolysaccharidosis type 1 (MPS 1) is a rare lysosomal storage disease belonging to the group of mucopolysaccharidoses. There are three variants, differing widely in their severity, with Hurler syndrome (57% of cases) being the most severe, Scheie syndrome (20% of cases) the mildest and Hurler-Scheie syndrome (23% of cases) giving an intermediate phenotype.

The diagnosis includes nothing.

The diagnosis excludes nothing.

It has no clarifying diagnoses.

The diagnosis is coded elsewhere:

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