What is ICD-10 code E75.3?

E75.3 is the ICD-10-CM diagnosis code for Sphingolipidosis, unspecified. A general category for inherited metabolic disorders involving abnormal accumulation of fatty substances in cells when the specific type has not been determined. It belongs to the ICD-10-CM chapter for endocrine, nutritional and metabolic diseases (e00-e89). E75.3 is a billable code, meaning it can be reported on a claim as a primary or secondary diagnosis when supported by documentation. E75.3 is part of the FY2026 ICD-10-CM code set published by the CMS National Center for Health Statistics and used on Medicare, Medicaid, and commercial claims in the United States.

Yes. E75.3 is a billable ICD-10-CM code that can be reported on professional and institutional claims as a primary or secondary diagnosis when the provider documentation supports the specific condition described. Billable codes in ICD-10-CM are coded to the highest level of specificity available for the documented diagnosis. E75.3 is current for fiscal year 2026 and remains in the active CMS ICD-10-CM code set published by the National Center for Health Statistics.

What is the V28 vs V24 difference for E75.3?

CMS phased in the V28 risk adjustment model over payment years 2024 (33%), 2025 (67%), and 2026 (100%), replacing V24. V28 consolidates HCC categories, removes some lower-acuity conditions, and recalibrates RAF weights across the entire model. E75.3 was a payment HCC under V24 (Other Significant Endocrine and Metabolic Disorders) but the V28 recalibration removed it from the risk adjustment model. Both models can still appear in legacy data and in payer-specific lookback periods.

What should coders know when reporting E75.3?

This is a non-specific code; use only when the type of sphingolipidosis cannot be determined from documentation Query the provider if additional diagnostic testing results are available to specify the disorder Because E75.3 maps to a payment HCC, the documentation must also satisfy MEAT criteria (Monitor, Evaluate, Assess, or Treat) for the encounter to count toward the patient's risk adjustment score.

2026 ICD-10-CM E75.3: Sphingolipidosis, unspecified

ICD-10-CM Code View

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Code lookupE75.3Effective periodFY2026 Apr Update (Apr 1-Sep 30)

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

E75.3

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

Sphingolipidosis, unspecified

A general category for inherited metabolic disorders involving abnormal accumulation of fatty substances in cells when the specific type has not been determined.

Buddy Insight

Sphingolipidosis, unspecified indicates a confirmed lysosomal storage disorder involving sphingolipid metabolism but without documentation specifying the exact type.

CMS-HCC V28

N/A

—

Not risk-adjusted for this model/period.

Not mapped

CMS-HCC V24

Mapped

HCC 23

Other Significant Endocrine a...

RAF 0.194

ACA/HHS

Mapped

HCC 27

HHS-HCC 27

Varies by metal level

ESRD/PACE

Mapped

HCC 23

Other Significant Endocrine a...

RAF 0.036

RXHCC

Mapped

HCC 41

Lysosomal Storage Disorders

RAF 3.081

Code Book Path

Official

E75Disorders of sphingolipid metabolism and other lipid storage disorders

E75.3Sphingolipidosis, unspecified

Inclusion Terms

Official

ICD-10-CM does not list inclusion terms for E75.3 in this effective period.

Excludes 2

Official

ICD-10-CM does not list Excludes 2 notes for E75.3 in this effective period.

Related Child Codes

Official

E75.0GM2 gangliosidosis

E75.1Other and unspecified gangliosidosis

E75.2Other sphingolipidosis

E75.4Neuronal ceroid lipofuscinosis

E75.5Other lipid storage disorders

Includes

Official

ICD-10-CM does not list Includes notes for E75.3 in this effective period.

Excludes 1

Official

mucolipidosis, types I-III (E77.0-E77.1)
Refsum's disease (G60.1)

Code First

Official

ICD-10-CM does not list Code First sequencing instructions for E75.3 in this effective period.

Use Additional

Official

ICD-10-CM does not list Use Additional Code instructions for E75.3 in this effective period.

Code Also

Official

ICD-10-CM does not list Code Also instructions for E75.3 in this effective period.

Buddy Documentation Tip

HCC Buddy guidance

Provider documentation confirming a sphingolipidosis diagnosis even if the specific subtype is unknown

Diagnostic workup results (enzyme levels, genetic testing, imaging, or biopsy) supporting the diagnosis

Clinical manifestations present (neurological symptoms, hepatosplenomegaly, skeletal abnormalities)

Current treatment plan and management approach

MEAT Support

HCC Buddy guidance

Provider documentation confirming a sphingolipidosis diagnosis even if the specific subtype is unknown

Diagnostic workup results (enzyme levels, genetic testing, imaging, or biopsy) supporting the diagnosis

Clinical manifestations present (neurological symptoms, hepatosplenomegaly, skeletal abnormalities)

Current treatment plan and management approach

Audit Caution

HCC Buddy guidance

Defaulting to unspecified code when the medical record contains enough detail to assign a specific sphingolipidosis code

Using this code for lipid storage disorders that are not sphingolipidoses (e.g., ceroid lipofuscinosis)

Not querying the provider for clarification when diagnostic results are available but uninterpreted in the note

Confusing this with general lipid metabolism disorders like hyperlipidemia (E78 category)

Common Mistakes

HCC Buddy guidance

E75.21 — Fabry disease: use when alpha-galactosidase A deficiency is confirmed

E75.22 — Gaucher disease: use when glucocerebrosidase deficiency is documented

E75.29 — Other sphingolipidosis: use when the type IS known but has no specific code

E75.5 — Other lipid storage disorders: for non-sphingolipid lipid storage conditions

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 E75.3 an HCC code?

Yes. E75.3 maps to Other Significant Endocrine and Metabolic Disorders under the V24 model but is not retained in V28.

Code: E75.3
Description: Sphingolipidosis, unspecified
HCC (V28): No CMS-HCC V28 mapping
RAF: —
Billable: Yes
Payment year: 2026

HCC Category Mapping

V24HCC 23, Other Significant Endocrine and Metabolic Disorders

0.194

ESRDHCC 23, Other Significant Endocrine and Metabolic Disorders

0.036

RxHCCHCC 41, Lysosomal Storage Disorders

3.081

Each model's RAF is its CMS base weight for that model's standard population, so 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 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.

Work E75.3 in the Code Book — tabular path, V28 RAF, and MEAT checklist →

MEAT Criteria for E75.3

For E75.3 to 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 E75.3 during that encounter, not just copy-forwarded from a problem list.

Coder workflow notes

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What This Code Means

E75.3 is the ICD-10-CM diagnosis code for sphingolipidosis, unspecified. A general category for inherited metabolic disorders involving abnormal accumulation of fatty substances in cells when the specific type has not been determined. E75.3 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 older CMS-HCC V24 model, E75.3 maps to Other Significant Endocrine and Metabolic Disorders (HCC 23) with a community, non-dual, aged base RAF weight of 0.194. 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.

This is a non-specific code; use only when the type of sphingolipidosis cannot be determined from documentation. Because E75.3 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 E75.3 sourced directly from the CMS-HCC risk adjustment model files and the CMS ICD-10-CM code set.

Coding Tips

•This is a non-specific code; use only when the type of sphingolipidosis cannot be determined from documentation
•Query the provider if additional diagnostic testing results are available to specify the disorder

Clinical Significance

Sphingolipidosis, unspecified indicates a confirmed lysosomal storage disorder involving sphingolipid metabolism but without documentation specifying the exact type. This diagnosis signals significant metabolic disease requiring ongoing specialist management and monitoring for progressive neurological and systemic complications.

Documentation Requirements

✓Provider documentation confirming a sphingolipidosis diagnosis even if the specific subtype is unknown
✓Diagnostic workup results (enzyme levels, genetic testing, imaging, or biopsy) supporting the diagnosis
✓Clinical manifestations present (neurological symptoms, hepatosplenomegaly, skeletal abnormalities)
✓Current treatment plan and management approach
✓Reason the specific type has not been determined (pending workup, atypical presentation)

Commonly Confused Codes

•E75.21: Fabry disease: use when alpha-galactosidase A deficiency is confirmed
•E75.22: Gaucher disease: use when glucocerebrosidase deficiency is documented
•E75.29: Other sphingolipidosis: use when the type IS known but has no specific code
•E75.5: Other lipid storage disorders: for non-sphingolipid lipid storage conditions
•E75.6: Lipid storage disorder, unspecified: broader category than sphingolipidosis