2026 ICD-10-CM Diagnosis Code E75.26Sulfatase deficiency

ICD-10-CM Codes›E00–E89›E70-E88›E75

ICD-10-CM E75.26
CMSSource: CMS FY 2026 ICD-10-CM dataset · Effective Oct 1, 2025 – Sep 30, 2026

E75.26 is a billable ICD-10-CM diagnosis code for sulfatase deficiency. It is valid on HIPAA claims for fiscal year 2026 (October 1, 2025 through September 30, 2026). Coders also document this condition as deficiency of sulfatase. 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
E75.26
Billable Status
Yes — Valid for Submission
Code Describes
Sulfatase deficiency
Short Description
Sulfatase deficiency
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 CodeE75.26Sulfatase deficiency

Approximate SynonymsGuidance

Alternate terms and clinical phrases that map to this code.

  • Deficiency of sulfatase

Tabular List NotesGuidance

Coding notes and annotation back-references applicable to this code.

Inclusion Terms

  • Multiple sulfatase deficiency (MSD)

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.

    • Deficiency, deficient
      • multiple sulfatase (MSD)
    • Deficiency, deficient
      • sulfatase
    • MSD(multiple sulfatase deficiency)

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

Genetic Brain Disorders

A genetic brain disorder is caused by a variation or a mutation in a gene. A variation is a different form of a gene. A mutation is a change in a gene. Genetic brain disorders affect the development and function of the brain.

Read the full article at MedlinePlus

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

Code History & ChangesHistory

Replacement E75.26 replaces the following previously assigned code(s):

  • E75.29 - Other sphingolipidosis
FY 2019AddedAdded to the ICD-10-CM code setEffective October 1, 2018.
FY 2020–2025No changes
FY 2026CurrentCurrent code set, no changesEffective October 1, 2025 through September 30, 2026.

Questions About E75.26Overview

Is E75.26 (Other sphingolipidosis) a billable code?

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

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.