What is ICD-10 code E75.248?

E75.248 is the ICD-10-CM diagnosis code for Other Niemann-Pick disease. Other Niemann-Pick disease refers to rare or atypical forms of this inherited metabolic disorder that do not fit into the standard type A, B, or C classifications. E75.248 is a billable code, meaning it can be reported on a claim as a primary or secondary diagnosis when supported by documentation. E75.248 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. E75.248 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. E75.248 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 E75.248?

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. E75.248 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 E75.248?

Use this code only when the specific type of Niemann-Pick disease cannot be determined or is documented as atypical Provide detailed documentation of the clinical presentation to support medical necessity and justify use of this code Because E75.248 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 E75.248: Other Niemann-Pick disease

ICD-10-CM Code View

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Code lookupE75.248Effective periodFY2026 Apr Update (Apr 1-Sep 30)

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

E75.248

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

Other Niemann-Pick disease

Other Niemann-Pick disease refers to rare or atypical forms of this inherited metabolic disorder that do not fit into the standard type A, B, or C classifications.

Buddy Insight

Other Niemann-Pick disease captures rare or atypical presentations of Niemann-Pick disease that do not fit the standard type A, B, C, D, or A/B classifications.

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

E75.2Other sphingolipidosis

E75.24Niemann-Pick disease

E75.248Other Niemann-Pick disease

Inclusion Terms

Official

Acid sphingomyelinase deficiency (ASMD)

Excludes 2

Official

ICD-10-CM does not list Excludes 2 notes for E75.248 in this effective period.

Related Child Codes

Official

E75.240Niemann-Pick disease type A

E75.241Niemann-Pick disease type B

E75.242Niemann-Pick disease type C

E75.243Niemann-Pick disease type D

E75.244Niemann-Pick disease type A/B

Includes

Official

ICD-10-CM does not list Includes notes for E75.248 in this effective period.

Excludes 1

Official

ICD-10-CM does not list Excludes 1 notes for E75.248 in this effective period.

Code First

Official

ICD-10-CM does not list Code First sequencing instructions for E75.248 in this effective period.

Use Additional

Official

ICD-10-CM does not list Use Additional Code instructions for E75.248 in this effective period.

Code Also

Official

ICD-10-CM does not list Code Also instructions for E75.248 in this effective period.

Buddy Documentation Tip

HCC Buddy guidance

Documented diagnosis of Niemann-Pick disease with atypical or unclassifiable features

Enzyme assay and genetic testing results

Detailed clinical presentation explaining why standard type classifications do not apply

Documentation of both neurological and visceral findings

MEAT Support

HCC Buddy guidance

Documented diagnosis of Niemann-Pick disease with atypical or unclassifiable features

Enzyme assay and genetic testing results

Detailed clinical presentation explaining why standard type classifications do not apply

Documentation of both neurological and visceral findings

Audit Caution

HCC Buddy guidance

Using this code as a default when the specific type simply hasn't been determined (use E75.249 instead)

Not documenting why the presentation is atypical or doesn't fit standard classifications

Confusing atypical Niemann-Pick with other lysosomal storage disorders

Failing to code organ-specific manifestations separately

Common Mistakes

HCC Buddy guidance

E75.249 — Niemann-Pick disease, unspecified: for cases where testing has not yet determined the type

E75.240-E75.244 — Specific Niemann-Pick types: use the appropriate specific code when the type is identified

E75.22 — Gaucher disease: different lysosomal storage disorder

E75.29 — Other sphingolipidosis: for non-Niemann-Pick sphingolipid disorders

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 E75.248 an HCC code?

Yes. E75.248 maps to Other Significant Endocrine and Metabolic Disorders under the V24 model but is not retained in V28.

Code: E75.248
Description: Other Niemann-Pick disease
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 E75.248 in the Code Book — tabular path, V28 RAF, and MEAT checklist →

MEAT Criteria for E75.248

For E75.248to 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 E75.248 during that encounter, not just copy-forwarded from a problem list.

Coder workflow notes

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What This Code Means

E75.248 is the ICD-10-CM diagnosis code for other niemann-pick disease. Other Niemann-Pick disease refers to rare or atypical forms of this inherited metabolic disorder that do not fit into the standard type A, B, or C classifications. E75.248 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, E75.248 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.

Use this code only when the specific type of Niemann-Pick disease cannot be determined or is documented as atypical. Because E75.248 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 E75.248 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 type of Niemann-Pick disease cannot be determined or is documented as atypical
•Provide detailed documentation of the clinical presentation to support medical necessity and justify use of this code

Clinical Significance

Other Niemann-Pick disease captures rare or atypical presentations of Niemann-Pick disease that do not fit the standard type A, B, C, D, or A/B classifications. This may include novel mutations with unique phenotypes or cases with unusual clinical features. Accurate coding of these atypical cases is important for rare disease registries and resource allocation.

Documentation Requirements

✓Documented diagnosis of Niemann-Pick disease with atypical or unclassifiable features
✓Enzyme assay and genetic testing results
✓Detailed clinical presentation explaining why standard type classifications do not apply
✓Documentation of both neurological and visceral findings
✓Genetic counseling documentation and family history

Commonly Confused Codes

•E75.249: Niemann-Pick disease, unspecified: for cases where testing has not yet determined the type
•E75.240-E75.244: Specific Niemann-Pick types: use the appropriate specific code when the type is identified
•E75.22: Gaucher disease: different lysosomal storage disorder
•E75.29: Other sphingolipidosis: for non-Niemann-Pick sphingolipid disorders
•E88.89: Other specified metabolic disorders: too general for documented Niemann-Pick