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ICD-10-CM Codes
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E00-E89
Endocrine, nutritional and metabolic diseases
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E70-E88
Metabolic disorders
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E75-
Disorders of sphingolipid metabolism and other lipid storage disorders
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2018 ICD-10-CM Diagnosis Code E75.21
Fabry (-Anderson) disease
2016 2017 2018 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 2018 edition of ICD-10-CM E75.21 became effective on October 1, 2017.
- 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
2018 ICD-10-CM Range E00-E89
Endocrine, nutritional and metabolic diseasesNote- 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)
- E70-E88
2018 ICD-10-CM Range E70-E88
Metabolic disordersType 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)
- E75
ICD-10-CM Diagnosis Code E75
Disorders of sphingolipid metabolism and other lipid storage disorders
2016 2017 2018 Non-Billable/Non-Specific Code
- E75.2
ICD-10-CM Diagnosis Code E75.2
Other sphingolipidosis
2016 2017 2018 Non-Billable/Non-Specific Code
Type 1 Excludes- adrenoleukodystrophy [Addison-Schilder] (E71.528)
- 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 v35.0):
- 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
ICD-10-CM Codes Adjacent To E75.21
E75.09 Other GM2 gangliosidosis
E75.1 Other and unspecified gangliosidosis
E75.10 Unspecified gangliosidosis
E75.2 Other sphingolipidosis
E75.21
Fabry (-Anderson) disease
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.
