E75.25 Metachromatic leukodystrophy

Billable ICD-10-CM code, FY2026. Acts as a Complication or Comorbidity (CC) as a secondary diagnosis.

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)

Applies from E75.2 Other sphingolipidosis

Excludes1 (never code together)

• adrenoleukodystrophy [Addison-Schilder] (E71.528)

Risk adjustment (HCC)

• RxHCC V08: HCC 41 — Lysosomal Storage Disorders
• ESRD V24: HCC 52

MS-DRG index

Defines the logic of DRG(s) 056-057 in MDC 01.

Approximate ICD-9-CM (GEMs)

• 330.0 Leukodystrophy (approximate)

Code annotations containing back-references to E75.25

Back-references to E75.2

• 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 E75.25

• Greenfield's disease › meaning › metachromatic leukodystrophy
• Leukodystrophy › metachromatic
• Leukoencephalopathy › metachromatic
• Scholz (-Bielchowsky-Henneberg) disease or syndrome

Nearby codes

• 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 (this code)
• E75.26 Sulfatase deficiency
• E75.27 Pelizaeus-Merzbacher disease
• E75.28 Canavan disease
• E75.29 Other sphingolipidosis
• E75.3 Sphingolipidosis, unspecified