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

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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

0

0

RAF 0

CMS-HCC V24

0

0

RAF 0

ACA/HHS

0

0

RAF 0

ESRD/PACE

0

0

RAF 0

RXHCC

HCC 43

RAF 0.0

Code Trumping

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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

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 E75.5 an HCC code?

No. E75.5 is a billable ICD-10-CM code but does not map to any HCC category in V28, V24, ESRD, or RxHCC.

HCC Category Mapping

RxHCCHCC 43, Other Significant Endocrine and Metabolic Disorders
0.000

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 E75.5

For E75.5to 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.

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 is a billable ICD-10-CM code but does not map to a payment HCC under the CMS-HCC V28, V24, ESRD, or RxHCC risk adjustment models. It can be reported on Medicare Advantage encounter data submissions but it does not contribute to a beneficiary's RAF score and therefore does not affect risk-adjusted payments to the plan.

E75.5 maps only to RxHCC 43 (Inflammatory Bowel Disease) with no RAF weight. It does not map to any CMS-HCC under V24 or V28 models, so it does not contribute to Medicare Advantage RAF score calculation. Its risk adjustment impact is limited to the prescription drug payment model.

HCC Buddy maintains structured V28 and V24 mapping, RAF weights, 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

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