2027 ICD-10-CM Diagnosis Code E75Disorders of sphingolipid metabolism and other lipid storage disorders

ICD-10-CM Codes›E00–E89›E70-E88›E75

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

E75 is a non-billable ICD-10-CM category code for disorders of sphingolipid metabolism and other lipid storage disorders, so it cannot be submitted on claims. Use a more specific code from this category instead, such as E75.00, E75.01, E75.02, and E75.09.

Code Identity

ICD-10-CM Code
E75
Billable Status
No — Non-Billable Category
Code Describes
Disorders of sphingolipid metabolism and other lipid storage disorders
Short Description
Disord of sphingolipid metab and oth lipid storage disorders
Chapter
E70-E88
Metabolic disorders

Code Classification

ChapterE00–E89Endocrine, nutritional and metabolic diseases
SectionE70-E88Metabolic disorders
CategoryE75Disorders of sphingolipid metabolism and other lipid storage disorders
This CodeE75Disorders of sphingolipid metabolism and other lipid storage disorders

Specific Coding for Disord of sphingolipid metab and oth lipid storage disordersOverview

Non-specific codes like E75 require more characters. Use one of these billable codes instead:

  • E75.0 for GM2 gangliosidosis

  • Use E75.00 for GM2 gangliosidosis, unspecified

  • Use E75.01 for Sandhoff disease

  • Use E75.02 for Tay-Sachs disease

  • Use E75.09 for Other GM2 gangliosidosis

  • E75.1 for Other and unspecified gangliosidosis

  • Use E75.10 for Unspecified gangliosidosis

  • Use E75.11 for Mucolipidosis IV

  • Use E75.19 for Other gangliosidosis

  • E75.2 for Other sphingolipidosis

  • Use E75.21 for Fabry (-Anderson) disease

  • Use E75.22 for Gaucher disease

  • Use E75.23 for Krabbe disease

  • E75.24 for Niemann-Pick disease

  • Use E75.25 for Metachromatic leukodystrophy

  • Use E75.26 for Sulfatase deficiency

  • Use E75.27 for Pelizaeus-Merzbacher disease

  • Use E75.28 for Canavan disease

  • Use E75.29 for Other sphingolipidosis

  • Use E75.3 for Sphingolipidosis, unspecified

  • Use E75.4 for Neuronal ceroid lipofuscinosis

  • Use E75.5 for Other lipid storage disorders

  • Use E75.6 for Lipid storage disorder, unspecified

Instructional NotesGuidance

Instructions from the official ICD-10-CM Tabular List that apply to E75: its own notes plus those printed at 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.

On E75 itself
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, its category, or a range that includes it.

Excludes1 1

These codes carry an Excludes1 note naming E75: 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.-)

Code First 2

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

  • G73.7 Myopathy in diseases classified elsewhere
    lipid storage disorders (E75.-)
  • H36 Retinal disorders in diseases classified elsewhere
    lipid storage disorders (E75.-)

Index to Diseases and InjuriesGuidance

Alphabetical index entries that point to this code.

Patient EducationClinical

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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 E75Overview

What is the ICD-10 code for disorders of sphingolipid metabolism and other lipid storage disorders?

E75 is the ICD-10-CM category for disorders of sphingolipid metabolism and other lipid storage disorders, but it is a non-billable header: claims need a more specific code from this category, listed on this page.

Is E75 a billable code?

No. This is a category header that groups the codes for disorders of sphingolipid metabolism and other lipid storage disorders, and headers cannot be submitted on claims. Claims for disorders of sphingolipid metabolism and other lipid storage disorders need a more specific code from this category, such as E75.00, E75.01, and E75.02.

Can E75 be reported with G60.1?

Not as a rule. The Excludes1 note on E75 lists "Refsum's disease (G60.1)". 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).