ICD-10-CM E75: Disorders of sphingolipid metabolism and other lipid storage disorders

ICD-10-CM Diagnosis Code (2025)

Official Description for E75

Disorders of sphingolipid metabolism and other lipid storage disorders

Coding Notes & Guidelines for E75

Excludes1: Not Coded Here
  • mucolipidosis, types I-III (E77.0-E77.1)
  • Refsum's disease (G60.1)
Excludes1: Not Coded Here

adrenoleukodystrophy [Addison-Schilder] (E71.528)

Inclusion Terms
  • Adult GM2 gangliosidosis
  • Juvenile GM2 gangliosidosis
Inclusion Terms

Gangliosidosis NOS

Inclusion Terms
  • GM1 gangliosidosis
  • GM3 gangliosidosis
Inclusion Terms

Acid sphingomyelinase deficiency (ASMD)

Inclusion Terms
  • Acid sphingomyelinase deficiency type A (ASMD type A)
  • Infantile neurovisceral acid sphingomyelinase deficiency
Inclusion Terms
  • Acid sphingomyelinase deficiency type B (ASMD type B)
  • Chronic visceral acid sphingomyelinase deficiency
Inclusion Terms
  • Acid sphingomyelinase deficiency type A/B (ASMD type A/B)
  • Chronic neurovisceral acid sphingomyelinase deficiency
Inclusion Terms

Acid sphingomyelinase deficiency (ASMD) NOS

Inclusion Terms

Multiple sulfatase deficiency (MSD)

Inclusion Terms
  • Farber's syndrome
  • Sulfatide lipidosis
Inclusion Terms
  • Batten disease
  • Bielschowsky-Jansky disease
  • Kufs disease
  • Spielmeyer-Vogt disease
Inclusion Terms
  • Cerebrotendinous cholesterosis [van Bogaert-Scherer-Epstein]
  • Wolman's disease

Child Codes (More Specific Codes for E75):

E75.0 - GM2 gangliosidosis E75.00 - GM2 gangliosidosis, unspecified E75.01 - Sandhoff disease E75.02 - Tay-Sachs disease E75.09 - Other GM2 gangliosidosis E75.1 - Other and unspecified gangliosidosis E75.10 - Unspecified gangliosidosis E75.11 - Mucolipidosis IV E75.19 - Other gangliosidosis E75.2 - Other sphingolipidosis E75.21 - Fabry (-Anderson) disease E75.22 - Gaucher disease E75.23 - Krabbe disease E75.24 - Niemann-Pick disease E75.240 - Niemann-Pick disease type A E75.241 - Niemann-Pick disease type B E75.242 - Niemann-Pick disease type C E75.243 - Niemann-Pick disease type D E75.244 - Niemann-Pick disease type A/B E75.248 - Other Niemann-Pick disease E75.249 - Niemann-Pick disease, unspecified E75.25 - Metachromatic leukodystrophy E75.26 - Sulfatase deficiency E75.27 - Pelizaeus-Merzbacher disease E75.28 - Canavan disease E75.29 - Other sphingolipidosis E75.3 - Sphingolipidosis, unspecified E75.4 - Neuronal ceroid lipofuscinosis E75.5 - Other lipid storage disorders E75.6 - Lipid storage disorder, unspecified

About ICD-10-CM Code E75 (Disorders of sphingolipid metabolism and other lipid storage disorders)

ICD-10-CM code E75 is a billable or specific ICD-10-CM code that can be used to indicate a diagnosis for reimbursement purposes. The 2025 edition of ICD-10-CM E75 became effective on October 1, 2024.

This page provides detailed information about E75 - Disorders of sphingolipid metabolism and other lipid storage disorders. It includes common terminology, coding guidelines such as "Includes," "Excludes1," and "Excludes2" notes, and information on required 7th characters. Utilizing this information correctly is essential for accurate medical coding and claims submission for conditions like Disorders of sphingolipid metabolism and other lipid storage disorders.