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E75.5 ICD-10-CM Code: Other lipid storage disorders

E75.5 is not a CMS-HCC payment code. MEAT criteria · RAF Calculator · HCC Buddy coding tools

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

E75.5

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

Other lipid storage disorders

A category for rare inherited metabolic disorders involving abnormal accumulation of lipids in the body that do not fit into other specific lipid storage disease categories.

Buddy the Bee presenting code insight

Buddy Insight

Other lipid storage disorders capture rare inherited conditions where lipids accumulate abnormally in cells but do not fall into the specific sphingolipidosis or ceroid lipofuscinosis categories.

CMS-HCC V28

N/A

Not mapped

CMS-HCC V24

Historical

Historical

Not used for CY2026 payment

ACA/HHS

N/A

Not mapped

ESRD/PACE

N/A

Not mapped

RXHCC

HCC 43

Code-level coefficient reference

Code Book Path

Official
E75Disorders of sphingolipid metabolism and other lipid storage disorders
E75.5Other lipid storage disorders

Inclusion Terms

Official
  • Cerebrotendinous cholesterosis [van Bogaert-Scherer-Epstein]
  • Wolman's disease

Excludes 2

Official

ICD-10-CM does not list Excludes 2 notes for E75.5 in this effective period.

Related Child Codes

Official
E75.0GM2 gangliosidosis
E75.1Other and unspecified gangliosidosis
E75.2Other sphingolipidosis
E75.3Sphingolipidosis, unspecified
E75.4Neuronal ceroid lipofuscinosis

Includes

Official

ICD-10-CM does not list Includes notes for E75.5 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 E75.5 in this effective period.

Use Additional

Official

ICD-10-CM does not list Use Additional Code instructions for E75.5 in this effective period.

Code Also

Official

ICD-10-CM does not list Code Also instructions for E75.5 in this effective period.

Buddy Documentation Tip

HCC Buddy guidance
Specific lipid storage disorder type documented (e.g., Wolman disease, cholesteryl ester storage disease, acid lipase deficiency)
Confirmatory diagnostic testing (enzyme assay, genetic testing, or tissue biopsy)
Affected organ systems and current clinical manifestations
Treatment plan including any enzyme replacement or dietary management

MEAT Support

HCC Buddy guidance
Specific lipid storage disorder type documented (e.g., Wolman disease, cholesteryl ester storage disease, acid lipase deficiency)
Confirmatory diagnostic testing (enzyme assay, genetic testing, or tissue biopsy)
Affected organ systems and current clinical manifestations
Treatment plan including any enzyme replacement or dietary management

Audit Caution

HCC Buddy guidance
Using E75.5 when a more specific lipid storage disorder code exists elsewhere in E75
Confusing lipid storage disorders (intracellular accumulation) with lipid metabolism disorders (circulating lipid abnormalities like hyperlipidemia)
Not querying for specificity when the provider documents a named lipid storage condition that may have its own code
Coding acid lipase deficiency as E75.5 when it may have a more specific classification

Common Mistakes

HCC Buddy guidance
E75.21-E75.29 — Sphingolipidoses: use when the lipid storage disorder specifically involves sphingolipids
E75.4 — Neuronal ceroid lipofuscinosis: a specific type with its own code, not 'other'
E75.6 — Lipid storage disorder, unspecified: use only when the type cannot be determined
E78.89 — Other lipoprotein metabolism disorders: for lipoprotein processing errors, not intracellular storage

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 E75.5 an HCC code?

E75.5 is not in the CMS-HCC V28 or V24 community payment model. E75.5 has a separate mapping under the Part D RxHCC model (HCC 43 (Pituitary, Adrenal Gland, and Other Endocrine and Metabolic Disorders)); the applicable result needs member context.

Code
E75.5
Description
Other lipid storage disorders
HCC (V28)
No CMS-HCC V28 mapping
RAF reference coefficient
Billable
Yes
Payment year
2026

HCC Category Mapping

RxHCCHCC 43, Pituitary, Adrenal Gland, and Other Endocrine and Metabolic 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 E75.5 in the Code Book — tabular path, V28 RAF reference, and MEAT checklist →

MEAT Criteria for E75.5

For E75.5 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 E75.5 during that encounter, not just copy-forwarded from a problem list.

Coder workflow notes

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What This Code Means

E75.5 is the ICD-10-CM diagnosis code for other lipid storage disorders. A category for rare inherited metabolic disorders involving abnormal accumulation of lipids in the body that do not fit into other specific lipid storage disease categories. E75.5 sits in the ICD-10-CM chapter for endocrine, nutritional and metabolic diseases (e00-e89), within the section covering metabolic disorders (e70-e88).

E75.5 has no mapping under the CMS-HCC V28 or V24 community payment models. E75.5 has a separate mapping under the Part D RxHCC model (HCC 43 (Pituitary, Adrenal Gland, and Other Endocrine and Metabolic Disorders)); the applicable result needs member context. Do not assign V28 risk adjustment value from this page; verify the applicable model and payment year before using this code for risk adjustment.

Use this code for lipid storage disorders not classified elsewhere.

HCC Buddy maintains structured V28 and V24 mapping, source-labeled coefficient references, and MEAT documentation criteria for E75.5 sourced directly from the CMS-HCC risk adjustment model files and the CMS ICD-10-CM code set.

Coding Tips

  • Use this code for lipid storage disorders not classified elsewhere
  • Ensure documentation specifies the type of lipid involved and affected organ systems

Clinical Significance

Other lipid storage disorders capture rare inherited conditions where lipids accumulate abnormally in cells but do not fall into the specific sphingolipidosis or ceroid lipofuscinosis categories. These conditions cause progressive organ damage and require complex, long-term management with significant healthcare resource consumption.

Documentation Requirements

  • Specific lipid storage disorder type documented (e.g., Wolman disease, cholesteryl ester storage disease, acid lipase deficiency)
  • Confirmatory diagnostic testing (enzyme assay, genetic testing, or tissue biopsy)
  • Affected organ systems and current clinical manifestations
  • Treatment plan including any enzyme replacement or dietary management
  • Provider assessment confirming this is a storage disorder, not a simple lipid metabolism issue

Commonly Confused Codes

  • E75.21-E75.29: Sphingolipidoses: use when the lipid storage disorder specifically involves sphingolipids
  • E75.4: Neuronal ceroid lipofuscinosis: a specific type with its own code, not 'other'
  • E75.6: Lipid storage disorder, unspecified: use only when the type cannot be determined
  • E78.89: Other lipoprotein metabolism disorders: for lipoprotein processing errors, not intracellular storage
  • E78.70: Disorder of bile acid and cholesterol metabolism, unspecified: for cholesterol metabolism pathway issues, not storage

Child Codes

Code Hierarchy

Work E75.5 in HCC Buddy

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