2026 ICD-10-CM Diagnosis Code E83.01Wilson's disease

ICD-10-CM Codes›E00–E89›E70-E88›E83

ICD-10-CM E83.01
CMSSource: CMS FY 2026 ICD-10-CM dataset · Effective Oct 1, 2025 – Sep 30, 2026

E83.01 is a billable ICD-10-CM diagnosis code for Wilson's disease. It is valid on HIPAA claims for fiscal year 2026 (October 1, 2025 through September 30, 2026). In AHRQ's Clinical Classifications Software (CCSR), this diagnosis falls under Other specified and unspecified nutritional and metabolic disorders.

Code Identity

ICD-10-CM Code
E83.01
Billable Status
Yes — Valid for Submission
Code Describes
Wilson's disease
Short Description
Wilson's disease
Same as the full description in the CMS dataset.
Parent Code
E83.0
Disorders of copper metabolism

Code Classification

ChapterE00–E89Endocrine, nutritional and metabolic diseases
SectionE70-E88Metabolic disorders
CategoryE83Disorders of mineral metabolism
This CodeE83.01Wilson's disease

Approximate SynonymsGuidance

Alternate terms and clinical phrases that map to this code.

  • Chorea co-occurrent and due to Wilson disease
  • Chorea due to heredodegenerative disorder
  • Chorea due to metabolic disorder
  • Dementia due to genetic disease
  • Dementia due to metabolic abnormality
  • Dementia due to Wilson disease
  • Disorder of copper metabolism
  • Disorder of liver due to disorder of mineral metabolism
  • Dystonia due to Wilson disease
  • Hypoparathyroidism due to Wilson disease
  • Westphal-Strumpell syndrome
  • Wilson's disease

Tabular List NotesGuidance

Coding notes and annotation back-references applicable to this code.

Code Also

  • associated Kayser Fleischer ring H18.04

Index to Diseases and InjuriesGuidance

Alphabetical index entries that point to this code.

External Cause of Injuries IndexGuidance

References for this code in the External Cause of Injuries Index.

    • Cirrhosis, cirrhotic(hepatic) (liver)
      • due to
        • hepatolenticular degeneration
    • Cirrhosis, cirrhotic(hepatic) (liver)
      • due to
        • Wilson's disease
    • Deficiency, deficient
      • ceruloplasmin (Wilson)
    • Degeneration, degenerative
      • hepatolenticular (Wilson's)
    • Degeneration, degenerative
      • lenticular (familial) (progressive) (Wilson's) (with cirrhosis of liver)
    • Degeneration, degenerative
      • Wilson's hepatolenticular
    • Dementia(degenerative (primary)) (persisting) (unspecified severity) (without behavioral disturbance, psychotic disturbance, mood disturbance, and anxiety)
      • in (due to)
        • hepatolenticular degeneration
    • Dementia(degenerative (primary)) (persisting) (unspecified severity) (without behavioral disturbance, psychotic disturbance, mood disturbance, and anxiety)
      • in (due to)
        • hepatolenticular degeneration
          • with behavioral disturbance
    • Disease, diseased
      • hepatolenticular
    • Disease, diseased
      • Kinnier Wilson's (hepatolenticular degeneration)
    • Disease, diseased
      • Wilson's (hepatolenticular degeneration)
    • Disorder(of)
      • metabolism NOS
        • copper
          • Wilson's disease
    • Disorder(of)
      • tubulo-interstitial (in)
        • Wilson's disease
    • Hepatolenticular degeneration
    • Kinnier Wilson's disease(hepatolenticular degeneration)
    • Lenticular degeneration, progressive
    • Pseudosclerosis(brain)
      • of Westphal (Strümpell)
    • Pyelonephritis
      • in (due to)
        • Wilson's disease
    • Strümpell-Westphal pseudosclerosis
    • Syndrome
      • amyostatic (Wilson's disease)
    • Syndrome
      • lenticular, progressive
    • Syndrome
      • Westphal-Strümpell
    • Syndrome
      • Wilson's (hepatolenticular degeneration)
    • Westphal-Strümpell syndrome
    • Wilson's
      • disease or syndrome
    • Wilson's
      • hepatolenticular degeneration

Clinical ClassificationClinical

AHRQ’s CCSR groups this code into broader clinical categories.

CCSR END016
Other specified and unspecified nutritional and metabolic disorders
Default principal diagnosis: inpatient Yes · outpatient Yes

Patient EducationClinical

Wilson Disease

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.

Read the full article at MedlinePlus

Courtesy of MedlinePlus, a service of the U.S. National Library of Medicine.

Convert E83.01 to ICD-9-CMHistory

The closest ICD-9-CM equivalents under the General Equivalence Mappings.

ICD-9-CM
275.1 Dis copper metabolism
Approximate The match is approximate rather than exact.

Code HistoryHistory

FY 2016AddedAdded to the ICD-10-CM code setEffective October 1, 2015, the first year of ICD-10-CM.
FY 2017–2025No changes
FY 2026CurrentCurrent code set, no changesEffective October 1, 2025 through September 30, 2026.

Questions About E83.01Overview

Is E83.01 (Disorders of copper metabolism) a billable code?

Yes. This is a billable ICD-10-CM code, specific enough to report Wilson's disease on HIPAA-covered claims from October 1, 2025 through September 30, 2026.

What is the ICD-9 equivalent of E83.01?

Under the General Equivalence Mappings, Wilson's disease converts to ICD-9-CM 275.1 (dis copper metabolism). The mapping is approximate, so confirm the match fits the documentation.

Footnotes

[1] Chronic - a chronic condition code indicates a condition lasting 12 months or longer and its effect on the patient based on one or both of the following criteria:

  • The condition results in the need for ongoing intervention with medical products,treatment, services, and special equipment
  • The condition places limitations on self-care, independent living, and social interactions.