DxCodebookFY2027 · Oct 1, 2026 – Sep 30, 2027

Category · FY2027

E75 Disorders of sphingolipid metabolism and other lipid storage disorders

  • Header · not billable

E75 is not billable. The category heads 26 billable codes in FY2027, grouped under 7 subdivisions.

Index → code

Where it sits

  1. Chapter 4 Endocrine, nutritional and metabolic diseases (E00-E89)
  2. E70-E88 Metabolic disorders (E70-E88)
  3. E75 Disorders of sphingolipid metabolism and other lipid storage disorders
Billable codes
26
Subdivisions
7
CC / MCC codes
14 / 0
HCC V28 codes
2
New FY2027
0
Index entries
104

Codes in E75

  1. E75.0
    GM2 gangliosidosis
    Header
  2. E75.00
    GM2 gangliosidosis, unspecified
    BillableCC
  3. E75.01
    Sandhoff disease
    BillableCC
  4. E75.02
    Tay-Sachs disease
    BillableCC
  5. E75.09
    Other GM2 gangliosidosis
    BillableCC
    Inclusion terms 2

    Inclusion terms

    • Adult GM2 gangliosidosis
    • Juvenile GM2 gangliosidosis
  6. E75.1
    Other and unspecified gangliosidosis
    Header
  7. E75.10
    Unspecified gangliosidosis
    BillableCC
    Inclusion terms 1

    Inclusion terms

    • Gangliosidosis NOS
  8. E75.11
    Mucolipidosis IV
    BillableCC
  9. E75.19
    Other gangliosidosis
    BillableCC
    Inclusion terms 2

    Inclusion terms

    • GM1 gangliosidosis
    • GM3 gangliosidosis
  10. E75.2
    Other sphingolipidosis
    Header
    Excludes1 1

    Excludes1

    • adrenoleukodystrophy [Addison-Schilder] (E71.528) E71.528
  11. E75.21
    Fabry (-Anderson) disease
    Billable49
  12. E75.22
    Gaucher disease
    Billable49
  13. E75.23
    Krabbe disease
    BillableCC
  14. E75.24
    Niemann-Pick disease
    Header
    Inclusion terms 1

    Inclusion terms

    • Acid sphingomyelinase deficiency (ASMD)
  15. E75.240
    Niemann-Pick disease type A
    Billable
    Inclusion terms 2

    Inclusion terms

    • Acid sphingomyelinase deficiency type A (ASMD type A)
    • Infantile neurovisceral acid sphingomyelinase deficiency
  16. E75.241
    Niemann-Pick disease type B
    Billable
    Inclusion terms 2

    Inclusion terms

    • Acid sphingomyelinase deficiency type B (ASMD type B)
    • Chronic visceral acid sphingomyelinase deficiency
  17. E75.242
    Niemann-Pick disease type C
    Billable
  18. E75.243
    Niemann-Pick disease type D
    Billable
  19. E75.244
    Niemann-Pick disease type A/B
    Billable
    Inclusion terms 2

    Inclusion terms

    • Acid sphingomyelinase deficiency type A/B (ASMD type A/B)
    • Chronic neurovisceral acid sphingomyelinase deficiency
  20. E75.248
    Other Niemann-Pick disease
    Billable
  21. E75.249
    Niemann-Pick disease, unspecified
    Billable
    Inclusion terms 1

    Inclusion terms

    • Acid sphingomyelinase deficiency (ASMD) NOS
  22. E75.25
    Metachromatic leukodystrophy
    BillableCC
  23. E75.26
    Sulfatase deficiency
    BillableCC
    Inclusion terms 1

    Inclusion terms

    • Multiple sulfatase deficiency (MSD)
  24. E75.27
    Pelizaeus-Merzbacher disease
    BillableCC
  25. E75.28
    Canavan disease
    BillableCC
  26. E75.29
    Other sphingolipidosis
    BillableCC
    Inclusion terms 2

    Inclusion terms

    • Farber's syndrome
    • Sulfatide lipidosis
  27. E75.3
    Sphingolipidosis, unspecified
    Billable
  28. E75.4
    Neuronal ceroid lipofuscinosis
    BillableCC
    Inclusion terms 4

    Inclusion terms

    • Batten disease
    • Bielschowsky-Jansky disease
    • Kufs disease
    • Spielmeyer-Vogt disease
  29. E75.5
    Other lipid storage disorders
    Billable
    Inclusion terms 2

    Inclusion terms

    • Cerebrotendinous cholesterosis [van Bogaert-Scherer-Epstein]
    • Wolman's disease
  30. E75.6
    Lipid storage disorder, unspecified
    Billable

CC/MCC: as a secondary diagnosis, unless the principal diagnosis is on the code’s PDX exclusion list (MS-DRG v44 Appendix C; list on the code card).

Sources of each column.

Instructions

At E75

Excludes1

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

Notes that also apply

Block E70-E88 instructions (Excludes1 5, Excludes2 1): printed on the block page.

Chapter 4 instructions (Notes 1, Excludes1 1): printed on the chapter 4 page.

Index entries

  • Sandhoff's disease → E75.01
  • Sachs' amaurotic familial idiocy or disease → E75.02
  • Sachs-Tay disease → E75.02
  • Tay-Sachs amaurotic familial idiocy or disease → E75.02
  • Gangliosidosis → E75.10
  • Anderson-Fabry disease → E75.21
  • Fabry (-Anderson) disease → E75.21
  • Sweeley-Klionsky disease → E75.21
  • Cerebroside lipidosis → E75.22
  • Gaucher's disease or splenomegaly (adult) (infantile) → E75.22
  • Niemann-Pick disease or syndrome → E75.249
  • Scholz (-Bielchowsky-Henneberg) disease or syndrome → E75.25
  • MSD (multiple sulfatase deficiency) → E75.26
  • Merzbacher-Pelizaeus disease → E75.27
  • Pelizaeus-Merzbacher disease → E75.27
  • Canavan disease → E75.28
  • Farber's disease or syndrome → E75.29
  • Myeloleukodystrophy → E75.29
  • Sphingolipidosis → E75.3
  • Sphingomyelinosis → E75.3
  • Amaurotic idiocy (infantile) (juvenile) (late) → E75.4
  • Batten (-Mayou) disease → E75.4
  • Bielschowsky (-Jansky) disease → E75.4
  • Ceroid-lipofuscinosis, neuronal → E75.4
  • Jansky-Bielschowsky amaurotic idiocy → E75.4
  • Kufs' disease → E75.4
  • Lipofuscinosis, neuronal (with ceroidosis) → E75.4
  • Spielmeyer-Vogt disease → E75.4
  • Vogt-Spielmeyer amaurotic idiocy or disease → E75.4
  • Van Bogaert-Scherer-Epstein disease or syndrome → E75.5
  • Wolman's disease → E75.5
  • Hyperlipidosis → E75.6
  • Lipidosis → E75.6
  • GangliosidosisGM2 → E75.00
  • Gangliosidosismucolipidosis IV → E75.11
  • MucolipidosisIV → E75.11
  • GangliosidosisGM1 → E75.19
  • GangliosidosisGM3 → E75.19
  • Angiokeratomacorporis diffusum → E75.21
  • Disease, diseasedSweeley-Klionsky → E75.21

64 more, longer entries are not listed; the search box finds them by word.

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