2027 ICD-10-CM Diagnosis Code E75.25Metachromatic leukodystrophy

ICD-10-CM Codes›E00–E89›E70-E88›E75

ICD-10-CM E75.25
CMSSource: CMS FY 2027 ICD-10-CM dataset · Effective Oct 1, 2026 – Sep 30, 2027

E75.25 is a billable ICD-10-CM diagnosis code for metachromatic leukodystrophy. It is valid on HIPAA claims for fiscal year 2027 (October 1, 2026 through September 30, 2027) and groups to MS-DRG 56 through 57. As a secondary diagnosis, it counts as a complication or comorbidity (CC) and moves an inpatient stay to a higher severity level within its MS-DRG family. It does not count, however, when the principal diagnosis is one of 24 closely related codes. In AHRQ's Clinical Classifications Software (CCSR), this diagnosis falls under Other nervous system disorders (often hereditary or degenerative) and Other specified and unspecified nutritional and metabolic disorders.

E75.25 no longer risk-adjusts for Medicare Advantage: it mapped to HCC 52 under the retired CMS-HCC V24 model through payment year 2025 but maps to no category in the live V28 model. It still risk-adjusts in the ESRD (V21) category 52, ESRD (V24) category 52, and RxHCC Part D (V08) category 41 for payment year 2027.

Code Identity

ICD-10-CM Code
E75.25
Billable Status
Yes — Valid for Submission
Code Describes
Metachromatic leukodystrophy
Short Description
Metachromatic leukodystrophy
Same as the full description in the CMS dataset.
Parent Code
E75.2
Other sphingolipidosis

Code Classification

ChapterE00–E89Endocrine, nutritional and metabolic diseases
SectionE70-E88Metabolic disorders
CategoryE75Disorders of sphingolipid metabolism and other lipid storage disorders
This CodeE75.25Metachromatic leukodystrophy

Medicare Risk Adjustment (HCC)Billing

E75.25 no longer risk-adjusts for Medicare Advantage: it maps to no payment category in the live CMS-HCC V28 model, although it still risk-adjusts in the other CMS models shown below.

CMS-HCC V28 (Medicare Advantage Payment Model)
Not mapped
Dropped in V28 see all codes that no longer risk-adjust
Prior Model (CMS-HCC V24)
HCC 52
V24 retired last contributed to a Medicare Advantage risk score in payment year 2025
Other CMS Models
ESRD (V21): HCC 52 · ESRD (V24): HCC 52
ESRD V21 weights: 0.045 dialysis, 0.000–0.328 functioning graft · ESRD V24 weights: 0.042 dialysis, 0.000–0.399 functioning graft
Part D (RxHCC)
RxHCC 41 — Lysosomal Storage Disorders
also risk-adjusts in the Part D prescription drug model (V08)

Source: CMS Payment Year 2027 risk adjustment mappings and model software. Weights are relative factors, not dollar amounts; a beneficiary's total RAF also includes demographics and interactions. Browse all CMS-HCC categories.

Approximate SynonymsGuidance

Alternate terms and clinical phrases that map to this code.

  • Arylsulfatase A deficiency
  • Dystonia due to metachromatic leucodystrophy
  • Metachromatic leukodystrophy
  • Metachromatic leukodystrophy due to deficiency of cerebroside sulfatase activator
  • Metachromatic leukodystrophy without arylsulfatase deficiency
  • Metachromatic leukodystrophy, adult type
  • Metachromatic leukodystrophy, congenital type
  • Metachromatic leukodystrophy, juvenile type
  • Metachromatic leukodystrophy, late infantile type
  • Pelizaeus Merzbacher like disease
  • Sphingolipid activator protein 1 deficiency

Instructional NotesGuidance

Instructions from the official ICD-10-CM Tabular List that apply to E75.25: its own notes plus those printed at E75.2, E75, block E70-E88, and Chapter 4. A note printed at a category, block or chapter applies to every code under it.

Excludes1

Not coded here: the excluded code is never reported together with this one, unless the two conditions are unrelated.

From E75.2 Other sphingolipidosis applies to 15 codes
  • adrenoleukodystrophy [Addison-Schilder] (E71.528)
From E75 Disorders of sphingolipid metabolism and other lipid storage disorders applies to 26 codes
From Block E70-E88 Metabolic disorders applies to 286 codes
  • androgen insensitivity syndrome (E34.5-)
  • congenital adrenal hyperplasia (E25.0)
  • hemolytic anemias attributable to enzyme disorders (D55.-)
  • Marfan syndrome (Q87.4-)
  • 5-alpha-reductase deficiency (E29.1)
From Chapter 4 (E00-E89) Endocrine, nutritional and metabolic diseases applies to 973 codes
  • transitory endocrine and metabolic disorders specific to newborn (P70-P74)

Excludes2

Not included here: the excluded condition is not part of this code, but a patient may have both, so both codes may be reported.

From Block E70-E88 Metabolic disorders applies to 286 codes
  • Ehlers-Danlos syndromes (Q79.6-)

Notes

General instructions on how these codes are used.

From Chapter 4 (E00-E89) Endocrine, nutritional and metabolic diseases applies to 973 codes
  • All neoplasms, whether functionally active or not, are classified in Chapter 2. Appropriate codes in this chapter (i.e. E05.8, E07.0, E16-E31, E34.-) may be used as additional codes to indicate either functional activity by neoplasms and ectopic endocrine tissue or hyperfunction and hypofunction of endocrine glands associated with neoplasms and other conditions classified elsewhere.

Source: CMS ICD-10-CM Tabular List. How to read instructional notes.

Referenced in Other NotesGuidance

Instructional notes printed at other codes that name E75.25, its category, or a range that includes it.

Excludes1 2

These codes carry an Excludes1 note naming E75.25: they are not reported together with it, unless the two conditions are unrelated.

  • R79 Other abnormal findings of blood chemistry
    specific findings indicating disorder of lipid metabolism (E75.-) names E75.-, which includes this code
  • E78 Disorders of lipoprotein metabolism and other lipidemias
    sphingolipidosis (E75.0-E75.3) names E75.0-E75.3, which includes this code

Code First 2

These codes say to code first a condition named in the note, and E75.25 is one of the conditions named.

  • G73.7 Myopathy in diseases classified elsewhere
    lipid storage disorders (E75.-) names E75.-, which includes this code
  • H36 Retinal disorders in diseases classified elsewhere
    lipid storage disorders (E75.-) names E75.-, which includes this code

Index to Diseases and InjuriesGuidance

Alphabetical index entries that point to this code.

Clinical ClassificationClinical

AHRQ’s CCSR groups this code into broader clinical categories.

CCSR NVS006
Other nervous system disorders (often hereditary or degenerative)
Default principal diagnosis: inpatient No · outpatient No
CCSR END016
Other specified and unspecified nutritional and metabolic disorders
Default principal diagnosis: inpatient Yes · outpatient Yes

Clinical InformationClinical

  • Leukodystrophy, Metachromatic

    an autosomal recessive metabolic disease caused by a deficiency of cerebroside-sulfatase leading to intralysosomal accumulation of cerebroside sulfate (sulfoglycosphingolipids) in the nervous system and other organs. pathological features include diffuse demyelination, and metachromatically-staining granules in many cell types such as the glial cells. there are several allelic and nonallelic forms with a variety of neurological symptoms.

Patient EducationClinical

Leukodystrophies

Leukodystrophies are a group of rare genetic disorders that affect the central nervous system (CNS). The CNS is made up of your brain and spinal cord. Leukodystrophies damage the white matter of your CNS. The white matter includes:

The full article covers:

  • What are leukodystrophies?
  • What causes leukodystrophies?
  • What are the symptoms of leukodystrophies?
  • How are leukodystrophies diagnosed?
  • What are the treatments for leukodystrophies?

Read the full article at MedlinePlus

Courtesy of MedlinePlus, a service of the U.S. National Library of Medicine.

Convert E75.25 to ICD-9-CMHistory

The closest ICD-9-CM equivalents under the General Equivalence Mappings.

ICD-9-CM
330.0 Leukodystrophy
Approximate The match is approximate rather than exact.

Code HistoryHistory

FY 2016AddedAdded to the ICD-10-CM code setEffective October 1, 2015, the first year of ICD-10-CM.
FY 2017–2026No changes
FY 2027CurrentCurrent code set, no changesEffective October 1, 2026 through September 30, 2027.

Questions About E75.25Overview

What is the ICD-10 code for metachromatic leukodystrophy?

The ICD-10-CM code for metachromatic leukodystrophy is E75.25 (sometimes written as E7525). It is billable on HIPAA-covered claims from October 1, 2026 through September 30, 2027.

Is E75.25 (Other sphingolipidosis) a billable code?

Yes. This is a billable ICD-10-CM code, specific enough to report metachromatic leukodystrophy on HIPAA-covered claims from October 1, 2026 through September 30, 2027.

What MS-DRG does E75.25 group to?

When metachromatic leukodystrophy is the principal diagnosis on an inpatient stay, it groups to MS-DRG 57, 56, with relative weights from 1.2835 to 2.2649 depending on complications. Higher weights mean higher Medicare reimbursement.

Is E75.25 a CC or MCC?

CMS lists E75.25 as a CC (complication or comorbidity) for FY 2027. Reported as a secondary diagnosis, it moves the inpatient stay to a higher-weighted DRG within its severity family. It does not count when the principal diagnosis is one of the 24 closely related codes in its exclusion list.

Can E75.25 be reported with E71.528?

Not as a rule. The Excludes1 note at E75.2, which applies to E75.25, lists "adrenoleukodystrophy [Addison-Schilder] (E71.528)". An Excludes1 note means the excluded code is never reported together with this one; the only exception is when the two conditions are unrelated to each other (Official Guidelines, Section I.A.12.a).

Does E75.25 risk-adjust for Medicare Advantage payment?

Not for Medicare Advantage. E75.25 mapped to HCC 52 in the retired CMS-HCC V24 model, which last determined payment in 2025, but it maps to no category in the live V28 model; see all codes that no longer risk-adjust. It still risk-adjusts in the ESRD (V21) category 52 (Dementia Without Complication), ESRD (V24) category 52 (Dementia Without Complication), and RxHCC Part D (V08) category 41 (Lysosomal Storage Disorders).