What is ICD-10 code E75.29?

E75.29 is the ICD-10-CM diagnosis code for Other sphingolipidosis. A group of rare inherited metabolic disorders involving abnormal accumulation of fatty substances in cells, causing progressive damage to the nervous system and other organs. It belongs to the ICD-10-CM chapter for endocrine, nutritional and metabolic diseases (e00-e89). E75.29 is a billable code, meaning it can be reported on a claim as a primary or secondary diagnosis when supported by documentation. E75.29 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. E75.29 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. E75.29 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 E75.29?

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. E75.29 was a payment HCC under V24 (Dementia Without Complication) 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 E75.29?

Use this code only when the specific sphingolipidosis type is not identified or documented Review medical record for diagnostic test results that may specify the exact lipid storage disorder Because E75.29 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 E75.29: Other sphingolipidosis

ICD-10-CM Code View

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Code lookupE75.29Effective periodFY2026 Apr Update (Apr 1-Sep 30)

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

E75.29

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

Other sphingolipidosis

A group of rare inherited metabolic disorders involving abnormal accumulation of fatty substances in cells, causing progressive damage to the nervous system and other organs.

Buddy Insight

Other sphingolipidosis represents rare inherited lysosomal storage disorders where specific sphingolipids accumulate abnormally in cells, causing progressive neurological and organ damage.

CMS-HCC V28

N/A

—

Not risk-adjusted for this model/period.

Not mapped

CMS-HCC V24

Mapped

HCC 52

Dementia Without Complication

RAF 0.346

ACA/HHS

Mapped

HCC 119

HHS-HCC 119

Varies by metal level

ESRD/PACE

Mapped

HCC 52

Dementia Without Complication

RAF 0.042

RXHCC

Mapped

HCC 41

Lysosomal Storage Disorders

RAF 3.081

Code Book Path

Official

E75Disorders of sphingolipid metabolism and other lipid storage disorders

E75.2Other sphingolipidosis

E75.29Other sphingolipidosis

Inclusion Terms

Official

Farber's syndrome
Sulfatide lipidosis

Excludes 2

Official

ICD-10-CM does not list Excludes 2 notes for E75.29 in this effective period.

Related Child Codes

Official

E75.21Fabry (-Anderson) disease

E75.22Gaucher disease

E75.23Krabbe disease

E75.24Niemann-Pick disease

E75.25Metachromatic leukodystrophy

Includes

Official

ICD-10-CM does not list Includes notes for E75.29 in this effective period.

Excludes 1

Official

adrenoleukodystrophy [Addison-Schilder] (E71.528)

Code First

Official

ICD-10-CM does not list Code First sequencing instructions for E75.29 in this effective period.

Use Additional

Official

ICD-10-CM does not list Use Additional Code instructions for E75.29 in this effective period.

Code Also

Official

ICD-10-CM does not list Code Also instructions for E75.29 in this effective period.

Buddy Documentation Tip

HCC Buddy guidance

Specific type of sphingolipidosis documented (e.g., Krabbe disease variant, metachromatic leukodystrophy variant) or statement that type is 'other' or atypical

Confirmatory diagnostic testing results (enzyme assay, genetic testing, or biopsy findings)

Current disease manifestations and affected organ systems

Treatment plan including enzyme replacement therapy, substrate reduction therapy, or supportive care

MEAT Support

HCC Buddy guidance

Specific type of sphingolipidosis documented (e.g., Krabbe disease variant, metachromatic leukodystrophy variant) or statement that type is 'other' or atypical

Confirmatory diagnostic testing results (enzyme assay, genetic testing, or biopsy findings)

Current disease manifestations and affected organ systems

Treatment plan including enzyme replacement therapy, substrate reduction therapy, or supportive care

Audit Caution

HCC Buddy guidance

Using E75.29 when a more specific sphingolipidosis code exists (e.g., Fabry, Gaucher, Krabbe, Niemann-Pick)

Coding from lab results alone without provider documentation confirming the diagnosis

Failing to query the provider when documentation says 'lipid storage disorder' without specifying sphingolipidosis type

Not recognizing that this code requires the sphingolipidosis to be specified as a type not elsewhere classified

Common Mistakes

HCC Buddy guidance

E75.21 — Fabry disease: a specific sphingolipidosis with alpha-galactosidase A deficiency, has its own code

E75.22 — Gaucher disease: specific sphingolipidosis with glucocerebrosidase deficiency, coded separately

E75.23 — Krabbe disease: specific globoid cell leukodystrophy, not classified under 'other'

E75.25 — Metachromatic leukodystrophy: has its own specific code, do not use E75.29

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 E75.29 an HCC code?

Yes. E75.29 maps to Dementia Without Complication under the V24 model but is not retained in V28.

Code: E75.29
Description: Other sphingolipidosis
HCC (V28): No CMS-HCC V28 mapping
RAF: —
Billable: Yes
Payment year: 2026

HCC Category Mapping

V24HCC 52, Dementia Without Complication

0.346

ESRDHCC 52, Dementia Without Complication

0.042

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 E75.29 in the Code Book — tabular path, V28 RAF, and MEAT checklist →

MEAT Criteria for E75.29

For E75.29 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 E75.29 during that encounter, not just copy-forwarded from a problem list.

Coder workflow notes

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What This Code Means

E75.29 is the ICD-10-CM diagnosis code for other sphingolipidosis. A group of rare inherited metabolic disorders involving abnormal accumulation of fatty substances in cells, causing progressive damage to the nervous system and other organs. E75.29 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, E75.29 maps to Dementia Without Complication (HCC 52) with a community, non-dual, aged base RAF weight of 0.346. 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.

Use this code only when the specific sphingolipidosis type is not identified or documented. Because E75.29 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 E75.29 sourced directly from the CMS-HCC risk adjustment model files and the CMS ICD-10-CM code set.

Coding Tips

•Use this code only when the specific sphingolipidosis type is not identified or documented
•Review medical record for diagnostic test results that may specify the exact lipid storage disorder

Clinical Significance

Other sphingolipidosis represents rare inherited lysosomal storage disorders where specific sphingolipids accumulate abnormally in cells, causing progressive neurological and organ damage. These conditions require lifelong management and significantly increase resource utilization due to their chronic, progressive nature and need for specialist care.

Documentation Requirements

✓Specific type of sphingolipidosis documented (e.g., Krabbe disease variant, metachromatic leukodystrophy variant) or statement that type is 'other' or atypical
✓Confirmatory diagnostic testing results (enzyme assay, genetic testing, or biopsy findings)
✓Current disease manifestations and affected organ systems
✓Treatment plan including enzyme replacement therapy, substrate reduction therapy, or supportive care
✓Provider assessment of disease status (stable, progressing, or in remission)

Commonly Confused Codes

•E75.21: Fabry disease: a specific sphingolipidosis with alpha-galactosidase A deficiency, has its own code
•E75.22: Gaucher disease: specific sphingolipidosis with glucocerebrosidase deficiency, coded separately
•E75.23: Krabbe disease: specific globoid cell leukodystrophy, not classified under 'other'
•E75.25: Metachromatic leukodystrophy: has its own specific code, do not use E75.29
•E75.3: Sphingolipidosis, unspecified: use only when no specific type is identified at all