2020 ICD-10-CM Diagnosis Code E75.21

Fabry (-Anderson) disease

    2016 2017 2018 2019 2020 Billable/Specific Code
  • E75.21 is a billable/specific ICD-10-CM code that can be used to indicate a diagnosis for reimbursement purposes.
  • The 2020 edition of ICD-10-CM E75.21 became effective on October 1, 2019.
  • This is the American ICD-10-CM version of E75.21 - other international versions of ICD-10 E75.21 may differ.
The following code(s) above E75.21 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 E75.21:
  • 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
  • E75
    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
    Type 1 Excludes
    Disorders of sphingolipid metabolism and other lipid storage disorders
  • E75.2
    ICD-10-CM Diagnosis Code E75.2

    Other sphingolipidosis

      2016 2017 2018 2019 2020 Non-Billable/Non-Specific Code
    Type 1 Excludes
    • adrenoleukodystrophy [Addison-Schilder] (E71.528)
    Other sphingolipidosis
Approximate Synonyms
  • Fabry's disease
  • Fabrys disease
Clinical Information
  • A rare x-linked inherited lysosomal storage disorder characterized by deficiency of the enzyme alpha-galactosidase a. It results in the accumulation of glycolipids in the blood vessels and tissues. Signs and symptoms include hypertension, cardiomyopathy, angiokeratomas, neuropathy, hypohidrosis, keratopathy, proteinuria, and renal failure.
  • An x-linked inherited metabolic disease caused by a deficiency of lysosomal alpha-galactosidase a. It is characterized by intralysosomal accumulation of globotriaosylceramide and other glycosphingolipids in blood vessels throughout the body leading to multi-system complications including renal, cardiac, cerebrovascular, and skin disorders.
  • X-linked lysosomal storage disease of glycosphingolipid catabolism, resulting from a deficiency of alpha-galactosidase a and leading to accumulation of ceramide trihexoside in the cardiovascular and renal systems.
ICD-10-CM E75.21 is grouped within Diagnostic Related Group(s) (MS-DRG v37.0):
  • 642 Inborn and other disorders of metabolism

Convert E75.21 to ICD-9-CM

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

Diagnosis Index entries containing back-references to E75.21:
  • Anderson-Fabry disease E75.21
  • Angiokeratoma - see Neoplasm, skin, benign
    • corporis diffusum E75.21
  • Degeneration, degenerative
    • brain (cortical) (progressive) G31.9
      ICD-10-CM Diagnosis Code G31.9

      Degenerative disease of nervous system, unspecified

        2016 2017 2018 2019 2020 Billable/Specific Code
      • in
        • Fabry-Anderson disease E75.21
  • Disease, diseased - see also Syndrome
    • Sweeley-Klionsky E75.21
  • Disorder (of) - see also Disease
    • glomerular (in) N05.9
      ICD-10-CM Diagnosis Code N05.9

      Unspecified nephritic syndrome with unspecified morphologic changes

        2016 2017 2018 2019 2020 Billable/Specific Code
      • Fabry's disease E75.21
  • Fabry E75.21 (-Anderson)
  • Glomerulonephritis N05.9
    - see also Nephritis
    ICD-10-CM Diagnosis Code N05.9

    Unspecified nephritic syndrome with unspecified morphologic changes

      2016 2017 2018 2019 2020 Billable/Specific Code
    • in (due to)
      • Fabry E75.21 (-Anderson)
  • Lipidosis E75.6
    ICD-10-CM Diagnosis Code E75.6

    Lipid storage disorder, unspecified

      2016 2017 2018 2019 2020 Billable/Specific Code
    • glycolipid E75.21
  • Sweeley-Klionsky disease E75.21

ICD-10-CM Codes Adjacent To E75.21
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
E75.21 Fabry (-Anderson) disease
E75.22 Gaucher disease
E75.23 Krabbe disease
E75.24 Niemann-Pick disease
E75.240 …… type A
E75.241 …… type B
E75.242 …… type C
E75.243 …… type D
E75.248 Other Niemann-Pick disease
E75.249 …… unspecified
E75.25 Metachromatic leukodystrophy

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