ICD-11 classes
14 Diseases of the skin
Genetic and developmental disorders affecting the skin
5C56.33 — Mucopolysaccharidosis type 6
ICD-11 5C56.33 — Mucopolysaccharidosis type 6
Mucopolysaccharidosis type 6 (MPS VI) is a lysosomal storage disease with progressive multisystem involvement, associated with a deficiency of arylsulfatase B (ASB) leading to the accumulation of dermatan sulfate. The disorder shows a wide spectrum of symptoms from slowly to rapidly progressing forms.
The diagnosis includes nothing.
The diagnosis excludes nothing.
It has no clarifying diagnoses.
The diagnosis is coded elsewhere: