E83.01 Wilson's disease

Billable ICD-10-CM code, FY2026.

Code also

• associated Kayser Fleischer ring (H18.04-)

Applies from E83 Disorders of mineral metabolism

Excludes1 (never code together)

• dietary mineral deficiency (E58-E61)
• parathyroid disorders (E20-E21)
• vitamin D deficiency (E55.-)

Risk adjustment (HCC)

• CMS-HCC V28: HCC 50 — Amyloidosis, Porphyria, and Other Specified Metabolic Disorders
• RxHCC V08: HCC 42 — Acromegaly and Other Endocrine and Metabolic Disorders

MS-DRG index

Defines the logic of DRG(s) 642 in MDC 10.

Approximate ICD-9-CM (GEMs)

• 275.1 Disorders of copper metabolism (approximate)

Code annotations containing back-references to E83.01

Back-references to E83.01

• Code First: F02 Dementia in other diseases classified elsewhere ("hepatolenticular degeneration (E83.01)")
• Code Also: H18.04 Kayser-Fleischer ring ("associated Wilson's disease (E83.01)")
• Code First: N16 Renal tubulo-interstitial disorders in diseases classified elsewhere ("Wilson's disease (E83.01)")

Back-references to E83

• Type 1 Excludes: E61 Deficiency of other nutrient elements ("disorders of mineral metabolism (E83.-)")
• Type 2 Excludes: G11 Hereditary ataxia ("metabolic disorders (E70-E88)")
• Code First: G63 Polyneuropathy in diseases classified elsewhere ("metabolic diseases (E70-E88)")
• Type 2 Excludes: G71 Primary disorders of muscles ("metabolic disorders (E70-E88)")
• Code First: H42 Glaucoma in diseases classified elsewhere ("specified metabolic disorder (E70-E88)")
• Type 2 Excludes: N25.0 Renal osteodystrophy ("metabolic disorders classifiable to E70-E88")
• Type 2 Excludes: N25.81 Secondary hyperparathyroidism of renal origin ("metabolic disorders classifiable to E70-E88")
• Type 1 Excludes: P59 Neonatal jaundice from other and unspecified causes ("jaundice due to inborn errors of metabolism (E70-E88)")
• Type 1 Excludes: R79.0 Abnormal level of blood mineral ("disorders of mineral metabolism (E83.-)")

Diagnosis Index entries for E83.01

• 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

Nearby codes

• E83 Disorders of mineral metabolism
• E83.0 Disorders of copper metabolism
• E83.00 Disorder of copper metabolism, unspecified
• E83.01 Wilson's disease (this code)
• 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