E75.3 Sphingolipidosis, unspecified

Billable ICD-10-CM code, FY2026.

Applies from E75 Disorders of sphingolipid metabolism and other lipid storage disorders

Excludes1 (never code together)

• mucolipidosis, types I-III (E77.0-E77.1)
• Refsum's disease (G60.1)

Risk adjustment (HCC)

• CMS-HCC V22: HCC 23 — Other Significant Endocrine and Metabolic Disorders
• RxHCC V08: HCC 41 — Lysosomal Storage Disorders
• ESRD V24: HCC 23

MS-DRG index

Defines the logic of DRG(s) 642 in MDC 10.

Approximate ICD-9-CM (GEMs)

• 272.7 Lipidoses (approximate)

Code annotations containing back-references to E75.3

Back-references to E75.3

• Type 1 Excludes: E78 Disorders of lipoprotein metabolism and other lipidemias ("sphingolipidosis (E75.0-E75.3)")

Back-references to E75

• Type 2 Excludes: G11 Hereditary ataxia ("metabolic disorders (E70-E88)")
• Code First: G63 Polyneuropathy in diseases classified elsewhere ("metabolic diseases (E70-E88)")
• Type 2 Excludes: G71 Primary disorders of muscles ("metabolic disorders (E70-E88)")
• Code First: G73.7 Myopathy in diseases classified elsewhere ("lipid storage disorders (E75.-)")
• Code First: H36 Retinal disorders in diseases classified elsewhere ("lipid storage disorders (E75.-)")
• Code First: H42 Glaucoma in diseases classified elsewhere ("specified metabolic disorder (E70-E88)")
• Type 2 Excludes: N25.0 Renal osteodystrophy ("metabolic disorders classifiable to E70-E88")
• Type 2 Excludes: N25.81 Secondary hyperparathyroidism of renal origin ("metabolic disorders classifiable to E70-E88")
• Type 1 Excludes: P59 Neonatal jaundice from other and unspecified causes ("jaundice due to inborn errors of metabolism (E70-E88)")
• Type 1 Excludes: R79 Other abnormal findings of blood chemistry ("specific findings indicating disorder of lipid metabolism (E75.-)")

Diagnosis Index entries for E75.3

• Degeneration, degenerative › brain (cortical) (progressive) › in › sphingolipidosis
• Sphingolipidosis
• Sphingomyelinosis

Nearby codes

• 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 (this code)
• E75.4 Neuronal ceroid lipofuscinosis
• E75.5 Other lipid storage disorders
• E75.6 Lipid storage disorder, unspecified