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E76.03 ICD-10-CM Code: Scheie's syndrome

E76.03 maps to CMS-HCC V28 49. A source-labeled RAF reference is available. Confirm the documented diagnosis and applicable coding requirements. MEAT criteria · RAF Calculator · free HCC coding tools

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Code lookupE76.03

FY 2026 Apr update / Endocrine, nutritional and metabolic diseases (E00-E89) / Metabolic disorders (E70-E88)

E76.03

Billable / SpecificICD-10-CMOfficial ICD-10-CMCodebook guidance

Scheie's syndrome

Scheie's syndrome is a rare inherited metabolic disorder where the body cannot properly break down certain complex sugars, leading to progressive organ damage and skeletal abnormalities.

Buddy the Bee presenting code insight

Buddy Insight

Scheie syndrome (MPS I-S) is the mildest form of mucopolysaccharidosis type I, characterized by normal intelligence, joint stiffness, corneal clouding, and aortic valve disease with near-normal lifespan.

OverviewRisk ModelsCode BookDocumentationAuditNotesEdition comparison

CMS-HCC V28

HCC 49

Coefficient HCC 49: 9.256 (Community Non-Dual Aged (CNA))

CMS-HCC V24

Historical

Historical

Not used for CY2026 payment

ACA/HHS

HCC 026

Code-level coefficient reference

ESRD/PACE

HCC 23

Code-level coefficient reference

RXHCC

HCC 41

Code-level coefficient reference

Code Book Path

Official
E76Disorders of glycosaminoglycan metabolismE76.0Mucopolysaccharidosis, type IE76.03Scheie's syndrome

Inclusion Terms

Official

No inclusion terms are included in this display for E76.03. Check the code and parent instructions in the Code Book.

Excludes 2

Official
  • Ehlers-Danlos syndromes (Q79.6-)Inherited from E70-E88

Related Codes

Official
E76.01Hurler's syndromeE76.02Hurler-Scheie syndrome

Includes

Official

No Includes notes are included in this display for E76.03. Check the code and parent instructions in the Code Book.

Excludes 1

Official
  • transitory endocrine and metabolic disorders specific to newborn (P70-P74)Inherited from E00-E89, E70-E88
  • androgen insensitivity syndrome (E34.5-)Inherited from E00-E89, E70-E88
  • congenital adrenal hyperplasia (E25.0)Inherited from E00-E89, E70-E88
  • hemolytic anemias attributable to enzyme disorders (D55.-)Inherited from E00-E89, E70-E88
  • Marfan syndrome (Q87.4-)Inherited from E00-E89, E70-E88
  • 5-alpha-reductase deficiency (E29.1)Inherited from E00-E89, E70-E88

Code First

Official

No Code First sequencing instructions are included in this display for E76.03. Check the code and parent instructions in the Code Book.

Use Additional

Official

No Use Additional Code instructions are included in this display for E76.03. Check the code and parent instructions in the Code Book.

Code Also

Official

No Code Also instructions are included in this display for E76.03. Check the code and parent instructions in the Code Book.

Buddy Documentation Tip

HCC Buddy guidance
Specific documentation of Scheie syndrome (MPS I-S) as the mild MPS I phenotype
Confirmatory enzyme assay showing alpha-L-iduronidase deficiency or IDUA gene mutation
Current clinical manifestations: joint contractures, corneal clouding, cardiac valve disease, carpal tunnel syndrome
Cognitive function assessment confirming normal intelligence (distinguishes from Hurler)

MEAT Support

HCC Buddy guidance
Specific documentation of Scheie syndrome (MPS I-S) as the mild MPS I phenotype
Confirmatory enzyme assay showing alpha-L-iduronidase deficiency or IDUA gene mutation
Current clinical manifestations: joint contractures, corneal clouding, cardiac valve disease, carpal tunnel syndrome
Cognitive function assessment confirming normal intelligence (distinguishes from Hurler)

Audit Caution

HCC Buddy guidance
Misclassifying a mild MPS I presentation as Hurler-Scheie or Hurler based on incomplete phenotype assessment
Coding only the manifestations (corneal opacity, joint stiffness, cardiac valve disease) without the underlying MPS I diagnosis
Using E76.3 (unspecified) when 'Scheie syndrome' is clearly documented
Not recognizing that Scheie syndrome was formerly classified as MPS V before reclassification as MPS I-S

Common Mistakes

HCC Buddy guidance
E76.01 — Hurler syndrome: severe MPS I with cognitive decline, much worse prognosis
E76.02 — Hurler-Scheie syndrome: intermediate severity with moderate systemic involvement
M77.10 — Lateral epicondylitis: joint stiffness in Scheie may mimic orthopedic conditions
H18.49 — Other corneal degeneration: corneal clouding in Scheie requires the metabolic code, not just the eye code

Current with CMS: FY2026 ICD-10-CM Apr 1 update (effective Apr 1 – Sep 30, 2026) · CMS-HCC V28, 100% phased in for payment year 2026. FY2027 code set already staged for October 1, 2026. How HCC Buddy stays current →

Is E76.03 an HCC code?

Yes. E76.03 (Scheie's syndrome) maps to HCC 49, Specified Lysosomal Storage Disorders under the CMS-HCC V28 risk adjustment model, with a source-labeled community non-dual aged reference coefficient of 9.256. Source-labeled code-level coefficients are references, not member totals. Actual contribution depends on complete member context, hierarchy and cleanup rules, interactions, and model year. HCC Buddy's RAF Calculator supports CMS-HCC V28 PY2026 and shows no score unless every required source and calculation check passes. It is billable for payment year 2026.

Coder answer: E76.03 is billable and maps to V28 HCC 49, Specified Lysosomal Storage Disorders. Open it in the Code Book for the tabular path, RAF, and MEAT checklist.

Code
E76.03
Description
Scheie's syndrome
HCC (V28)
HCC 49 — Specified Lysosomal Storage Disorders
RAF reference coefficient
9.256
Billable
Yes
Payment year
2026

HCC Category Mapping

V28HCC 49, Specified Lysosomal Storage Disorders
9.256
ESRDHCC 23, Other Significant Endocrine and Metabolic Disorders
Not separately weighted
RxHCCHCC 41, Lysosomal Storage Disorders
Not separately weighted

These are source-labeled model coefficients, not member totals. A category may still be removed by hierarchy or model cleanup rules. Weights are not directly comparable across models: CMS-HCC V28 and V24 use Community, Non-Dual, Aged; ESRD uses the dialysis continuing-enrollee model; RxHCC is the Part D continuing-enrollee, non-low-income, aged weight (a larger scale than CMS-HCC). ACA/HHS has no single weight — it varies by metal level. Actual per-patient RAF contribution depends on member context, hierarchy and cleanup rules, interactions, and the model year used by the payer. V28 is the CMS-HCC model phased in over payment years 2024–2026; V24 remains available for historical review.

Work E76.03 in the Code Book — tabular path, V28 RAF reference, and MEAT checklist →

MEAT review for E76.03

For E76.03, confirm that the documentation supports the diagnosis and meets the applicable coding, encounter, program and payer requirements. MEAT (Monitor, Evaluate, Assess, or Treat) is a review mnemonic, not a universal CMS coding rule.

  • MMonitor: signs, symptoms, disease progression, or lab trending documented in the note
  • EEvaluate: test results, medication response, or physical findings reviewed by the provider
  • AAssess: explicit mention in the assessment or plan with acknowledgment of status
  • TTreat: medication, referral, procedure, therapy, or counseling tied to the diagnosis

Coder workflow notes

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What This Code Means

E76.03 is the ICD-10-CM diagnosis code for scheie's syndrome. Scheie's syndrome is a rare inherited metabolic disorder where the body cannot properly break down certain complex sugars, leading to progressive organ damage and skeletal abnormalities. E76.03 sits in the ICD-10-CM chapter for endocrine, nutritional and metabolic diseases (e00-e89), within the section covering metabolic disorders (e70-e88).

Under the CMS-HCC V28 risk adjustment model, E76.03 maps to Specified Lysosomal Storage Disorders (HCC 49) with a source-labeled community, non-dual, aged reference coefficient of 9.256. No V24 mapping is shown for E76.03; use the applicable model and payment year when reviewing the V28 mapping. For CY2026 non-PACE Medicare Advantage, CMS uses 100% of the 2024 CMS-HCC model (V28). PACE uses a separate model blend. Source-labeled code-level coefficients are references, not member totals. Actual contribution depends on complete member context, hierarchy and cleanup rules, interactions, and model year. HCC Buddy's RAF Calculator supports CMS-HCC V28 PY2026 and shows no score unless every required source and calculation check passes.

Scheie's syndrome is a specific form of mucopolysaccharidosis type I; do not use the unspecified MPS I code if this diagnosis is documented. For E76.03, confirm that the documentation supports the diagnosis and meets the applicable coding, encounter, program and payer requirements. MEAT (Monitor, Evaluate, Assess, or Treat) is a review mnemonic, not a universal CMS coding rule. When documentation is ambiguous, coders should issue a provider query rather than assume the highest-specificity variant.

HCC Buddy maintains structured V28 and V24 mapping, source-labeled coefficient references, and MEAT documentation criteria for E76.03 sourced directly from the CMS-HCC risk adjustment model files and the CMS ICD-10-CM code set.

Coding Tips

  • •Scheie's syndrome is a specific form of mucopolysaccharidosis type I; do not use the unspecified MPS I code if this diagnosis is documented
  • •Document any associated complications such as cardiac valve disease, corneal clouding, or skeletal deformities for complete coding

Clinical Significance

Scheie syndrome (MPS I-S) is the mildest form of mucopolysaccharidosis type I, characterized by normal intelligence, joint stiffness, corneal clouding, and aortic valve disease with near-normal lifespan. Though milder than Hurler, it still requires ongoing specialist monitoring and often enzyme replacement therapy to prevent progressive joint and cardiac complications.

Documentation Requirements

  • ✓Specific documentation of Scheie syndrome (MPS I-S) as the mild MPS I phenotype
  • ✓Confirmatory enzyme assay showing alpha-L-iduronidase deficiency or IDUA gene mutation
  • ✓Current clinical manifestations: joint contractures, corneal clouding, cardiac valve disease, carpal tunnel syndrome
  • ✓Cognitive function assessment confirming normal intelligence (distinguishes from Hurler)
  • ✓Treatment plan including enzyme replacement therapy and symptom management

Commonly Confused Codes

  • •E76.01: Hurler syndrome: severe MPS I with cognitive decline, much worse prognosis
  • •E76.02: Hurler-Scheie syndrome: intermediate severity with moderate systemic involvement
  • •M77.10: Lateral epicondylitis: joint stiffness in Scheie may mimic orthopedic conditions
  • •H18.49: Other corneal degeneration: corneal clouding in Scheie requires the metabolic code, not just the eye code

Child Codes

E76.01Hurler's syndrome
HCC 49
E76.02Hurler-Scheie syndrome
HCC 49
E76.03Scheie's syndrome
HCC 49

Code Hierarchy

└E76Disorders of glycosaminoglycan metabolism└E76.0Mucopolysaccharidosis, type I└E76.03Scheie's syndrome
└E76.03Scheie's syndrome

Also searched as

  • E76 03
  • E7603

For E76.03, confirm that the documentation supports the diagnosis and meets the applicable coding, encounter, program and payer requirements. MEAT (Monitor, Evaluate, Assess, or Treat) is a review mnemonic, not a universal CMS coding rule.

E76.03 maps to CMS-HCC V28 category 49, Specified Lysosomal Storage Disorders. See the ICD-10 to HCC mapping hub for how the V28 crosswalk works. The mapping identifies a payment HCC category for E76.03. Review its source-labeled HCC coefficient above, check the RAF Calculator with complete member context for CMS-HCC V28 PY2026, and confirm the documentation the chart needs before the code is submitted. HCC Buddy shows no RAF score unless every required source and calculation check passes.

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Open E76.03 in the Code Book for the full Index-to-Tabular path, MEAT checklist, and V28 HCC mapping, or in the Encoder to code from a keyword search. Pro includes 7 days to try everything, no card required.

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