E76.03 ICD-10-CM Code: Scheie's syndrome
HCC Buddy Code Card
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FY 2026 Apr update / Endocrine, nutritional and metabolic diseases (E00-E89) / Metabolic disorders (E70-E88)
E76.03
Billable / SpecificICD-10-CMOfficial ICD-10-CMCodebook guidanceScheie'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 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.
CMS-HCC V28
MappedHCC 49
RAF 0.226
CMS-HCC V24
MappedHCC 23
RAF 0.230
ACA/HHS
00
RAF 0
ESRD/PACE
MappedHCC 23
RAF 0.0
RXHCC
MappedHCC 41
RAF 0.0
Code Trumping
Basket needed
Code Book Path
Inclusion Terms
OfficialICD-10-CM does not list inclusion terms for E76.03 in this effective period.
Excludes 2
OfficialICD-10-CM does not list Excludes 2 notes for E76.03 in this effective period.
Related Child Codes
Includes
OfficialICD-10-CM does not list Includes notes for E76.03 in this effective period.
Excludes 1
OfficialICD-10-CM does not list Excludes 1 notes for E76.03 in this effective period.
Code First
OfficialICD-10-CM does not list Code First sequencing instructions for E76.03 in this effective period.
Use Additional
OfficialICD-10-CM does not list Use Additional Code instructions for E76.03 in this effective period.
Code Also
OfficialICD-10-CM does not list Code Also instructions for E76.03 in this effective period.
Buddy Documentation Tip
MEAT Support
Audit Caution
Common Mistakes
Last updated: FY2026 ICD-10-CM Apr update, Apr 1, 2026 through Sep 30, 2026. CMS-HCC V28 is 100% phased in for payment year 2026.
Is E76.03 an HCC code?
Yes. E76.03 maps to Lysosomal Storage Disorders under the CMS-HCC V28 risk adjustment model (and Other Significant Endocrine and Metabolic Disorders under V24).
HCC Category Mapping
RAF weights shown are the community, non-dual, aged base weights from the CMS risk adjustment model file. Actual per-patient RAF contribution depends on member segment, interactions, and the model year used by the payer. V28 is the CMS-HCC model phased in over payment years 2024–2026; V24 remains in use during the transition and for historical data.
MEAT Criteria for E76.03
For E76.03to count as a valid HCC diagnosis in a given encounter, the provider's documentation must show MEAT: Monitor, Evaluate, Assess, or Treat. A diagnosis from a prior year does not carry forward automatically, it has to be re-documented and supported each calendar year.
- 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
Only one of M/E/A/T is required to support the code, but the documentation must be specific enough to show that the provider actually addressed E76.03 during that encounter, not just copy-forwarded from a problem list.
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 Lysosomal Storage Disorders (HCC 49) with a community, non-dual, aged base RAF weight of 0.226. Under the older CMS-HCC V24 model, E76.03 maps to Other Significant Endocrine and Metabolic Disorders (HCC 23) with a community, non-dual, aged base RAF weight of 0.230. V28 is the CMS-HCC risk adjustment model that reached 100% phase-in for payment year 2026, replacing V24 which was used during the PY2024–PY2025 transition.
Scheie's syndrome is a specific form of mucopolysaccharidosis type I; do not use the unspecified MPS I code if this diagnosis is documented. Because E76.03 maps to a payment HCC, the provider's documentation must satisfy MEAT criteria (Monitor, Evaluate, Assess, or Treat) for the encounter to count toward the patient's Medicare Advantage risk adjustment score. 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, RAF weights, 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