2020 ICD-10-CM Diagnosis Code E75

Disorders of sphingolipid metabolism and other lipid storage disorders

    2016 2017 2018 2019 2020 Non-Billable/Non-Specific Code
  • E75 should not be used for reimbursement purposes as there are multiple codes below it that contain a greater level of detail.
  • Short description: Disord of sphingolipid metab and oth lipid storage disorders
  • The 2020 edition of ICD-10-CM E75 became effective on October 1, 2019.
  • This is the American ICD-10-CM version of E75 - other international versions of ICD-10 E75 may differ.
Type 1 Excludes
Type 1 Excludes Help
A type 1 excludes note is a pure excludes. It means "not coded here". A type 1 excludes note indicates that the code excluded should never be used at the same time as E75. A type 1 excludes note is for used for when two conditions cannot occur together, such as a congenital form versus an acquired form of the same condition.
  • mucolipidosis, types I-III (
    ICD-10-CM Diagnosis Code E77.0

    Defects in post-translational modification of lysosomal enzymes

      2016 2017 2018 2019 2020 Billable/Specific Code
    Applicable To
    • Mucolipidosis II [I-cell disease]
    • Mucolipidosis III [pseudo-Hurler polydystrophy]
    E77.0-
    ICD-10-CM Diagnosis Code E77.1

    Defects in glycoprotein degradation

      2016 2017 2018 2019 2020 Billable/Specific Code
    Applicable To
    • Aspartylglucosaminuria
    • Fucosidosis
    • Mannosidosis
    • Sialidosis [mucolipidosis I]
    E77.1
    )
  • Refsum's disease (
    ICD-10-CM Diagnosis Code G60.1

    Refsum's disease

      2016 2017 2018 2019 2020 Billable/Specific Code
    Applicable To
    • Infantile Refsum disease
    G60.1
    )
The following code(s) above E75 contain annotation back-references
Annotation Back-References
In this context, annotation back-references refer to codes that contain:
  • Applicable To annotations, or
  • Code Also annotations, or
  • Code First annotations, or
  • Excludes1 annotations, or
  • Excludes2 annotations, or
  • Includes annotations, or
  • Note annotations, or
  • Use Additional annotations
that may be applicable to E75:
  • E00-E89
    2020 ICD-10-CM Range E00-E89

    Endocrine, nutritional and metabolic diseases

    Note
    • All neoplasms, whether functionally active or not, are classified in Chapter 2. Appropriate codes in this chapter (i.e. E05.8, E07.0, E16-E31, E34.-) may be used as additional codes to indicate either functional activity by neoplasms and ectopic endocrine tissue or hyperfunction and hypofunction of endocrine glands associated with neoplasms and other conditions classified elsewhere.
    Type 1 Excludes
    • transitory endocrine and metabolic disorders specific to newborn (P70-P74)
    Endocrine, nutritional and metabolic diseases
  • E70-E88
    2020 ICD-10-CM Range E70-E88

    Metabolic disorders

    Type 1 Excludes
    • androgen insensitivity syndrome (E34.5-)
    • congenital adrenal hyperplasia (E25.0)
    • Ehlers-Danlos syndrome (Q79.6-)
    • hemolytic anemias attributable to enzyme disorders (D55.-)
    • Marfan's syndrome (Q87.4)
    • 5-alpha-reductase deficiency (E29.1)
    Metabolic disorders
Code History
  • 2016 (effective 10/1/2015): New code (first year of non-draft ICD-10-CM)
  • 2017 (effective 10/1/2016): No change
  • 2018 (effective 10/1/2017): No change
  • 2019 (effective 10/1/2018): No change
  • 2020 (effective 10/1/2019): No change
Code annotations containing back-references to E75:
  • Code First: G73.7
    , H36
    ICD-10-CM Diagnosis Code G73.7

    Myopathy in diseases classified elsewhere

      2016 2017 2018 2019 2020 Billable/Specific Code Manifestation Code
    Code First
    • underlying disease, such as:
    • hyperparathyroidism (E21.0, E21.3)
    • hypoparathyroidism (E20.-)
    • glycogen storage disease (E74.0)
    • lipid storage disorders (E75.-)
    Type 1 Excludes
    • myopathy in:
    • rheumatoid arthritis (M05.32)
    • sarcoidosis (D86.87)
    • scleroderma (M34.82)
    • sicca syndrome [Sjögren] (M35.03)
    • systemic lupus erythematosus (M32.19)
    ICD-10-CM Diagnosis Code H36

    Retinal disorders in diseases classified elsewhere

      2016 2017 2018 2019 2020 Billable/Specific Code Manifestation Code
    Code First
    • underlying disease, such as:
    • lipid storage disorders (E75.-)
    • sickle-cell disorders (D57.-)
    Type 1 Excludes
  • Type 1 Excludes: R79
    ICD-10-CM Diagnosis Code R79

    Other abnormal findings of blood chemistry

      2016 2017 2018 2019 2020 Non-Billable/Non-Specific Code
    Type 1 Excludes
    • asymptomatic hyperuricemia (E79.0)
    • hyperglycemia NOS (R73.9)
    • hypoglycemia NOS (E16.2)
    • neonatal hypoglycemia (P70.3-P70.4)
    • specific findings indicating disorder of amino-acid metabolism (E70-E72)
    • specific findings indicating disorder of carbohydrate metabolism (E73-E74)
    • specific findings indicating disorder of lipid metabolism (E75.-)
    Use Additional
    • code to identify any retained foreign body, if applicable (Z18.-)
ICD-10-CM Codes Adjacent To E75
E74.2 Disorders of galactose metabolism
E74.20 …… unspecified
E74.21 Galactosemia
E74.29 Other disorders of galactose metabolism
E74.3 Other disorders of intestinal carbohydrate absorption
E74.31 Sucrase-isomaltase deficiency
E74.39 Other disorders of intestinal carbohydrate absorption
E74.4 Disorders of pyruvate metabolism and gluconeogenesis
E74.8 Other specified disorders of carbohydrate metabolism
E74.9 Disorder of carbohydrate metabolism, unspecified
E75 Disorders of sphingolipid metabolism and other lipid storage disorders
E75.0 GM2 gangliosidosis
E75.00 …… 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

Reimbursement claims with a date of service on or after October 1, 2015 require the use of ICD-10-CM codes.