2026 ICD-10-CM Diagnosis Code E75.23Krabbe disease

ICD-10-CM Codes›E00–E89›E70-E88›E75

ICD-10-CM E75.23
CMSSource: CMS FY 2026 ICD-10-CM dataset · Effective Oct 1, 2025 – Sep 30, 2026

E75.23 is a billable ICD-10-CM diagnosis code for krabbe disease. It is valid on HIPAA claims for fiscal year 2026 (October 1, 2025 through September 30, 2026). Coders also document this condition as atypical Krabbe disease due to saposin A deficiency. 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.

Code Identity

ICD-10-CM Code
E75.23
Billable Status
Yes — Valid for Submission
Code Describes
Krabbe disease
Short Description
Krabbe disease
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 CodeE75.23Krabbe disease

Approximate SynonymsGuidance

Alternate terms and clinical phrases that map to this code.

  • Atypical Krabbe disease due to saposin A deficiency
  • Galactocerebroside beta-galactosidase deficiency - early onset
  • Galactosylceramide beta-galactosidase deficiency
  • Globoid cell leukodystrophy, late-onset

Index to Diseases and InjuriesGuidance

Alphabetical index entries that point to this code.

External Cause of Injuries IndexGuidance

References for this code in the External Cause of Injuries Index.

    • Krabbe's
      • disease
    • Sclerosis, sclerotic
      • brain (generalized) (lobular)
        • Krabbe's

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

  • Other Sphingolipidosis|Other sphingolipidosis

    evidence of other sphingolipidosis not specified elsewhere.

  • Sphingolipidosis

    an inherited metabolic disorder that affects the metabolism of the spinhgolipids. representative examples include gaucher disease, tay-sachs disease, and niemann-pick disease.

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 E75.23 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 2017–2025No changes
FY 2026CurrentCurrent code set, no changesEffective October 1, 2025 through September 30, 2026.

Questions About E75.23Overview

Is E75.23 (Other sphingolipidosis) a billable code?

Yes. This is a billable ICD-10-CM code, specific enough to report krabbe disease on HIPAA-covered claims from October 1, 2025 through September 30, 2026.

What is the ICD-9 equivalent of E75.23?

Under the General Equivalence Mappings, krabbe disease converts to ICD-9-CM 330.0 (leukodystrophy). The mapping is approximate, so confirm the match fits the documentation.

Footnotes

[1] Chronic - a chronic condition code indicates a condition lasting 12 months or longer and its effect on the patient based on one or both of the following criteria:

  • The condition results in the need for ongoing intervention with medical products,treatment, services, and special equipment
  • The condition places limitations on self-care, independent living, and social interactions.