ICD-10-CM 2027
ICD-10 code for Scheie's syndrome
From the official ICD-10-CM alphabetic index entry “Scheie's syndrome”. Page updated September 29, 2026.
About coding Scheie's syndrome
The ICD-10-CM code for Scheie's syndrome is E76.03 (Scheie's syndrome).
Within E76.0 (Mucopolysaccharidosis, type I), E76.03 is specifically for Scheie's syndrome. Related codes cover Hurler's syndrome (E76.01) and Hurler-Scheie syndrome (E76.02).
As a secondary diagnosis it is a CC (complication or comorbidity), which can move an inpatient stay into the "with CC" MS-DRG of its family, unless the principal diagnosis excludes it.
In MS-DRG v44.0, it is part of the grouping logic for DRG 642 (Inborn and Other Disorders of Metabolism, relative weight 1.3219), in MDC 10 (Endocrine, Nutritional and Metabolic Diseases and Disorders), as a principal or secondary diagnosis. The DRG a claim lands in depends on the principal diagnosis, procedures and any CC/MCC secondary diagnoses.
Common questions about Scheie's syndrome ICD-10 codes
What is the ICD-10 code for Scheie's syndrome?
- E76.03 — Scheie's syndrome.
Is E76.03 billable?
- Yes. E76.03 is a billable/specific ICD-10-CM code valid for FY2027 (October 1, 2026 – September 30, 2027).
Can E76.03 be used as a principal diagnosis?
- Yes. The Medicare Code Editor doesn't restrict E76.03 as a principal diagnosis.
Is E76.03 a CC or MCC?
- E76.03 is a CC (complication or comorbidity) under MS-DRG v44.0, unless excluded by the principal diagnosis.