ICD-10 Code E76.03 – Scheie’s syndrome (2026): Diagnosis, Symptoms & Billing Guide
2026 ICD-10-CM Diagnosis Code E76.03 – Scheie’s syndrome
What it is
E76.03 identifies Scheie syndrome, a rare inherited lysosomal storage disorder in the mucopolysaccharidosis I spectrum. It causes buildup of glycosaminoglycans and progressive multisystem disease.
Clinical signs
Typical findings include corneal clouding, joint stiffness, coarse facial features, and skeletal changes. Cardiac, airway, and hearing involvement may occur; clinical features vary, so refer to documentation.
When to use this code
Use E76.03 when the provider documents Scheie syndrome or mucopolysaccharidosis I, Scheie type. Apply it for confirmed diagnoses in problem lists, encounters, or specialist assessments. If the record only says “MPS I” without type, check documentation before coding.
Do not use for
Do not use this code for Hurler syndrome, Hurler-Scheie syndrome, or unspecified mucopolysaccharidosis I. If the chart does not clearly identify Scheie syndrome, do not assume it.
Coding tip
Code the exact MPS I subtype documented; when the subtype is unclear, query the provider.