ICD-10 Code E76.02 – Hurler-Scheie Syndrome (2026): Diagnosis, Symptoms & Billing Guide
2026 ICD-10-CM Diagnosis Code E76.02 – Hurler-Scheie syndrome
What it is
E76.02 identifies Hurler-Scheie syndrome, a mucopolysaccharidosis disorder with features between Hurler and Scheie syndromes. Use it for the documented diagnosis, not for nonspecific storage disease or carrier status.
Clinical signs
Clinical features vary; refer to documentation. Common findings include skeletal abnormalities, joint stiffness, coarse facial features, corneal clouding, and possible organ involvement. Confirm the diagnosis is specifically Hurler-Scheie syndrome.
When to use this code
Use E76.02 when the provider documents Hurler-Scheie syndrome or an equivalent confirmed diagnosis. It may also apply when the chart clearly identifies a mucopolysaccharidosis type I intermediate phenotype. If the record only says “MPS” or another subtype, check documentation.
Do not use for
Do not use this code for Hurler syndrome, Scheie syndrome, or unspecified mucopolysaccharidosis without confirmation. Avoid it when the diagnosis is only suspected or when documentation is incomplete.
Coding tip
Code E76.02 only when the provider names Hurler-Scheie syndrome explicitly; otherwise, query for the exact mucopolysaccharidosis type.