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  2. ICD-10-CM
  3. Ch. E00-E89
  4. Sec. E70-E88
  5. Category E75
Category E75

Disorders of sphingolipid metabolism and other lipid storage disorders

Clinical Classification Guidelines

Specific Diagnosis Codes

GM2 gangliosidosis
E75.0
GM2 gangliosidosis, unspecified
E75.00
Sandhoff disease
E75.01
Tay-Sachs disease
E75.02
Other GM2 gangliosidosis
E75.09
Other and unspecified gangliosidosis
E75.1
Unspecified gangliosidosis
E75.10
Mucolipidosis IV
E75.11
Other gangliosidosis
E75.19
Other sphingolipidosis
E75.2
Fabry (-Anderson) disease
E75.21
Gaucher disease
E75.22
Krabbe disease
E75.23
Niemann-Pick disease
E75.24
Niemann-Pick disease type A
E75.240
Niemann-Pick disease type B
E75.241
Niemann-Pick disease type C
E75.242
Niemann-Pick disease type D
E75.243
Niemann-Pick disease type A/B
E75.244
Other Niemann-Pick disease
E75.248
Niemann-Pick disease, unspecified
E75.249
Metachromatic leukodystrophy
E75.25
Sulfatase deficiency
E75.26
Pelizaeus-Merzbacher disease
E75.27
Canavan disease
E75.28
Other sphingolipidosis
E75.29
Sphingolipidosis, unspecified
E75.3
Neuronal ceroid lipofuscinosis
E75.4
Other lipid storage disorders
E75.5
Lipid storage disorder, unspecified
E75.6
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Data updated for October 2025 release.

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