2021 ICD-10-CM Diagnosis Code E83.01

Wilson's disease

    2016 2017 2018 2019 2020 2021 Billable/Specific Code
  • E83.01 is a billable/specific ICD-10-CM code that can be used to indicate a diagnosis for reimbursement purposes.
  • The 2021 edition of ICD-10-CM E83.01 became effective on October 1, 2020.
  • This is the American ICD-10-CM version of E83.01 - other international versions of ICD-10 E83.01 may differ.
Code Also
Code Also Help
A code also note instructs that 2 codes may be required to fully describe a condition but the sequencing of the two codes is discretionary, depending on the severity of the conditions and the reason for the encounter.
  • associated Kayser Fleischer ring (
    ICD-10-CM Diagnosis Code H18.04

    Kayser-Fleischer ring

      2016 2017 2018 2019 2020 2021 Non-Billable/Non-Specific Code
    Code Also
    • associated Wilson's disease (E83.01)
The following code(s) above E83.01 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 E83.01:
  • E00-E89
    2021 ICD-10-CM Range E00-E89

    Endocrine, nutritional and metabolic diseases

    • 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
    2021 ICD-10-CM Range E70-E88

    Metabolic disorders

    Type 1 Excludes
    • androgen insensitivity syndrome (E34.5-)
    • congenital adrenal hyperplasia (E25.0)
    • Ehlers-Danlos syndromes (Q79.6-)
    • hemolytic anemias attributable to enzyme disorders (D55.-)
    • Marfan's syndrome (Q87.4)
    • 5-alpha-reductase deficiency (E29.1)
    Metabolic disorders
  • E83
    ICD-10-CM Diagnosis Code E83

    Disorders of mineral metabolism

      2016 2017 2018 2019 2020 2021 Non-Billable/Non-Specific Code
    Type 1 Excludes
    • dietary mineral deficiency (E58-E61)
    • parathyroid disorders (E20-E21)
    • vitamin D deficiency (E55.-)
    Disorders of mineral metabolism
Approximate Synonyms
  • Bilateral kayser-fleischer rings
  • Kayser-fleischer ring, both eyes
  • Left kayser-fleischer ring
  • Left kayser-fleischer ring (eye condition)
  • Right kayser-fleischer ring
  • Wilsons disease
Clinical Information
  • A rare autosomal recessive disease characterized by the deposition of copper in the brain; liver; cornea; and other organs. It is caused by defects in the atp7b gene encoding copper-transporting atpase 2 (ec, also known as the wilson disease protein. The overload of copper inevitably leads to progressive liver and neurological dysfunction such as liver cirrhosis; tremor; ataxia and intellectual deterioration. Hepatic dysfunction may precede neurologic dysfunction by several years.
  • A rare autosomal recessive inherited disorder caused by mutations in the atp7b gene. It is characterized by copper accumulation in the tissues, particularly brain and liver. It results in liver failure, neurologic, and psychotic manifestations.
  • Rare autosomal recessive disease characterized by the deposition of copper in the brain, liver, cornea and other organs; clinical features include liver cirrhosis, liver failure, splenomegaly, tremor, bradykinesia, dysarthria, chorea, muscle rigidity, kayser-fleischer rings (pigmented corneal lesions), ataxia and intellectual deterioration; hepatic dysfunction may precede neurologic dysfunction by several years.
  • Wilson disease is a rare inherited disorder that prevents your body from getting rid of extra copper. You need a small amount of copper from food to stay healthy. Too much copper is poisonous.normally, your liver releases extra copper into bile, a digestive fluid. With wilson disease, the copper builds up in your liver, and it releases the copper directly into your bloodstream. This can cause damage to your brain, kidneys, and eyes.wilson disease is present at birth, but symptoms usually start between ages 5 and 35. It first attacks the liver, the central nervous system or both. The most characteristic sign is a rusty brown ring around the cornea of the eye. A physical exam and laboratory tests can diagnose it.treatment is with drugs to remove the extra copper from your body. You need to take medicine and follow a low-copper diet for the rest of your life. Don't eat shellfish or liver, as these foods may contain high levels of copper. At the beginning of treatment, you'll also need to avoid chocolate, mushrooms, and nuts. Have your drinking water checked for copper content and don't take multivitamins that contain copper.with early detection and proper treatment, you can enjoy good health.
ICD-10-CM E83.01 is grouped within Diagnostic Related Group(s) (MS-DRG v38.0):
  • 642 Inborn and other disorders of metabolism

Convert E83.01 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
  • 2021 (effective 10/1/2020): No change
Code annotations containing back-references to E83.01:
  • Code Also: H18.04
    ICD-10-CM Diagnosis Code H18.04

    Kayser-Fleischer ring

      2016 2017 2018 2019 2020 2021 Non-Billable/Non-Specific Code
    Code Also
    • associated Wilson's disease (E83.01)

Diagnosis Index entries containing back-references to E83.01:
  • Cirrhosis, cirrhotic (hepatic) (liver) K74.60
    ICD-10-CM Diagnosis Code K74.60

    Unspecified cirrhosis of liver

      2016 2017 2018 2019 2020 2021 Billable/Specific Code
    Applicable To
    • Cirrhosis (of liver) NOS
    • due to
      • hepatolenticular degeneration E83.01
      • Wilson's disease E83.01
  • Deficiency, deficient
    • ceruloplasmin E83.01 (Wilson)
  • Degeneration, degenerative
    • hepatolenticular E83.01 (Wilson's)
    • lenticular E83.01 (familial) (progressive) (Wilson's) (with cirrhosis of liver)
    • Wilson's hepatolenticular E83.01
  • Dementia (degenerative (primary)) (old age) (persisting) F03.90
    ICD-10-CM Diagnosis Code F03.90

    Unspecified dementia without behavioral disturbance

      2016 2017 2018 2019 2020 2021 Billable/Specific Code Adult Dx (15-124 years)
    Applicable To
    • Dementia NOS
  • Disease, diseased - see also Syndrome
    • hepatolenticular E83.01
    • Kinnier Wilson's E83.01 (hepatolenticular degeneration)
    • Wilson's E83.01 (hepatolenticular degeneration)
  • Disorder (of) - see also Disease
    • metabolism NOS E88.9
      ICD-10-CM Diagnosis Code E88.9

      Metabolic disorder, unspecified

        2016 2017 2018 2019 2020 2021 Billable/Specific Code
      • copper E83.00
        ICD-10-CM Diagnosis Code E83.00

        Disorder of copper metabolism, unspecified

          2016 2017 2018 2019 2020 2021 Billable/Specific Code
        • Wilson's disease E83.01
    • tubulo-interstitial (in)
      • Wilson's disease E83.01
  • Hepatolenticular degeneration E83.01
  • Kinnier Wilson's disease E83.01 (hepatolenticular degeneration)
  • Lenticular degeneration, progressive E83.01
  • Pseudosclerosis (brain)
    • of Westphal E83.01 (Strümpell)
  • Pyelonephritis - see also Nephritis, tubulo-interstitial
    • in (due to)
      • Wilson's disease E83.01
  • Strümpell-Westphal pseudosclerosis E83.01
  • Syndrome - see also Disease
    • amyostatic E83.01 (Wilson's disease)
    • lenticular, progressive E83.01
    • Westphal-Strümpell E83.01
    • Wilson's E83.01 (hepatolenticular degeneration)
  • Westphal-Strümpell syndrome E83.01
  • Wilson's
    • disease or syndrome E83.01
    • hepatolenticular degeneration E83.01

ICD-10-CM Codes Adjacent To E83.01
E80.21 Acute intermittent (hepatic) porphyria
E80.29 Other porphyria
E80.3 Defects of catalase and peroxidase
E80.4 Gilbert syndrome
E80.5 Crigler-Najjar syndrome
E80.6 Other disorders of bilirubin metabolism
E80.7 Disorder of bilirubin metabolism, unspecified
E83 Disorders of mineral metabolism
E83.0 Disorders of copper metabolism
E83.00 Disorder of copper metabolism, unspecified
E83.01 Wilson's disease
E83.09 Other disorders of copper metabolism
E83.1 Disorders of iron metabolism
E83.10 Disorder of iron metabolism, unspecified
E83.11 Hemochromatosis
E83.110 Hereditary hemochromatosis
E83.111 …… due to repeated red blood cell transfusions
E83.118 Other hemochromatosis
E83.119 …… unspecified
E83.19 Other disorders of iron metabolism
E83.2 Disorders of zinc metabolism

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