2019 ICD-10-CM Diagnosis Code E75.5

Other lipid storage disorders

    2016 2017 2018 2019 Billable/Specific Code
  • E75.5 is a billable/specific ICD-10-CM code that can be used to indicate a diagnosis for reimbursement purposes.
  • The 2019 edition of ICD-10-CM E75.5 became effective on October 1, 2018.
  • This is the American ICD-10-CM version of E75.5 - other international versions of ICD-10 E75.5 may differ.
Applicable To
  • Cerebrotendinous cholesterosis [van Bogaert-Scherer-Epstein]
  • Wolman's disease
The following code(s) above E75.5 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 E75.5:
  • E00-E89
    2019 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
    2019 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 Non-Billable/Non-Specific Code
    Type 1 Excludes
    Disorders of sphingolipid metabolism and other lipid storage disorders
Approximate Synonyms
  • Cholesterol ester storage disease
  • Neutral lipid storage disorder
  • Tendon xanthoma
  • Triglyceride storage disease with ichthyosis
  • Xanthoma tendinosum
Clinical Information
  • A very rare, autosomal recessive inherited lysosomal storage disease caused by mutations in the lipa gene. Signs and symptoms appear in infancy and include developmental delay, vomiting, abdominal distention, hepatosplenomegaly, jaundice, anemia, steatorrhea, and calcification of the adrenal glands.
  • Severe infantile form of inherited lysosomal lipid storage disease due to deficiency of acid lipase; results in accumulation of neutral lipids, particularly cholesterol esters, within cells.
  • The severe infantile form of inherited lysosomal lipid storage diseases due to deficiency of acid lipase (sterol esterase). It is characterized by the accumulation of neutral lipids, particularly cholesterol esters in leukocytes, fibroblasts, and hepatocytes. It is also known as wolman's xanthomatosis and is an allelic variant of cholesterol ester storage disease.
ICD-10-CM E75.5 is grouped within Diagnostic Related Group(s) (MS-DRG v36.0):
  • 642 Inborn and other disorders of metabolism

Convert E75.5 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
Code annotations containing back-references to E75.5:
  • Type 1 Excludes: E78.2
    ICD-10-CM Diagnosis Code E78.2

    Mixed hyperlipidemia

      2016 2017 2018 2019 Billable/Specific Code
    Applicable To
    • Broad- or floating-betalipoproteinemia
    • Combined hyperlipidemia NOS
    • Elevated cholesterol with elevated triglycerides NEC
    • Fredrickson's hyperlipoproteinemia, type IIb or III
    • Hyperbetalipoproteinemia with prebetalipoproteinemia
    • Hypercholesteremia with endogenous hyperglyceridemia
    • Hyperlipidemia, group C
    • Tubo-eruptive xanthoma
    • Xanthoma tuberosum
    Type 1 Excludes
    • cerebrotendinous cholesterosis [van Bogaert-Scherer- Epstein] (E75.5)
    • familial combined hyperlipidemia (E78.49)

Diagnosis Index entries containing back-references to E75.5:
  • Cholesterolosis, cholesterosis (gallbladder) K82.4
    ICD-10-CM Diagnosis Code K82.4

    Cholesterolosis of gallbladder

      2016 2017 2018 2019 Billable/Specific Code
    Applicable To
    • Strawberry gallbladder
    Type 1 Excludes
    • cholesterolosis of gallbladder with cholecystitis (K81.-)
    • cholesterolosis of gallbladder with cholelithiasis (K80.-)
    • cerebrotendinous E75.5
  • Disease, diseased - see also Syndrome
    • lipid-storage E75.6
      ICD-10-CM Diagnosis Code E75.6

      Lipid storage disorder, unspecified

        2016 2017 2018 2019 Billable/Specific Code
      • specified NEC E75.5
    • triglyceride-storage E75.5
  • Disorder (of) - see also Disease
    • lipid
      • storage E75.6
        ICD-10-CM Diagnosis Code E75.6

        Lipid storage disorder, unspecified

          2016 2017 2018 2019 Billable/Specific Code
        • specified NEC E75.5
  • Hyperlipidosis E75.6
    ICD-10-CM Diagnosis Code E75.6

    Lipid storage disorder, unspecified

      2016 2017 2018 2019 Billable/Specific Code
    • hereditary NEC E75.5
  • Lipidosis E75.6
    ICD-10-CM Diagnosis Code E75.6

    Lipid storage disorder, unspecified

      2016 2017 2018 2019 Billable/Specific Code
    • cholesterol E75.5 (cerebral)
  • Van Bogaert-Scherer-Epstein disease or syndrome E75.5
  • Wolman's disease E75.5
  • Xanthoma(s), xanthomatosis (primary) (familial) (hereditary) E75.5
    • cerebrotendinous E75.5
    • cutaneotendinous E75.5
    • joint E75.5
    • tendon E75.5 (sheath)

ICD-10-CM Codes Adjacent To E75.5
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
E75.26 Sulfatase deficiency
E75.29 Other sphingolipidosis
E75.3 Sphingolipidosis, unspecified
E75.4 Neuronal ceroid lipofuscinosis
E75.5 Other lipid storage disorders
E75.6 Lipid storage disorder, unspecified
E76 Disorders of glycosaminoglycan metabolism
E76.0 Mucopolysaccharidosis, type I
E76.01 Hurler's syndrome
E76.02 Hurler-Scheie syndrome
E76.03 Scheie's syndrome
E76.1 Mucopolysaccharidosis, type II
E76.2 Other mucopolysaccharidoses
E76.21 Morquio mucopolysaccharidoses
E76.210 Morquio A mucopolysaccharidoses

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