Disorders of porphyrin and bilirubin metabolism (E80) ICD-10-CM
The E80 code range covers disorders of porphyrin and bilirubin metabolism with 12 ICD-10-CM diagnosis codes. 10 of them are billable and valid for claim submission in fiscal year 2027, and the category headers group them but cannot themselves be billed.
Includes
This note appears immediately under a three character code title to further define, or give examples of, the content of the category.
- defects of catalase and peroxidase
Codes in the E80 Range 12 codes · 10 billable
- E80 Disorders of porphyrin and bilirubin metabolismNon-billable
- E80.0 Hereditary erythropoietic porphyria
- E80.1 Porphyria cutanea tarda
- E80.2 Other and unspecified porphyriaNon-billable
- E80.20 Unspecified porphyria
- 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
Clinical Terms in This Code Range
Definitions from the National Library of Medicine for conditions coded in the E80 range.
Acatalasia
A rare autosomal recessive disorder resulting from the absence of CATALASE activity. Though usually asymptomatic, a syndrome of oral ulcerations and gangrene may be present.
Coproporphyria, Hereditary
An autosomal dominant porphyria that is due to a deficiency of COPROPORPHYRINOGEN OXIDASE in the LIVER, the sixth enzyme in the 8-enzyme biosynthetic pathway of HEME. Clinical features include both neurological symptoms and cutaneous lesions. Patients excrete increased levels of porphyrin precursors, 5-AMINOLEVULINATE and COPROPORPHYRINS.
Crigler-Najjar Syndrome
A familial form of congenital hyperbilirubinemia transmitted as an autosomal recessive trait. It is characterized by icterus and brain damage caused by a glucuronyl transferase deficiency in the liver and faulty bilirubin conjugation.
Gilbert Disease
A benign familial disorder, transmitted as an autosomal dominant trait. It is characterized by low-grade chronic hyperbilirubinemia with considerable daily fluctuations of the bilirubin level.
Porphyria Cutanea Tarda
An autosomal dominant or acquired porphyria due to a deficiency of UROPORPHYRINOGEN DECARBOXYLASE in the LIVER. It is characterized by photosensitivity and cutaneous lesions with little or no neurologic symptoms. Type I is the acquired form and is strongly associated with liver diseases and hepatic toxicities caused by alcohol or estrogenic steroids. Type II is the familial form.
Porphyria, Acute Intermittent
An autosomal dominant porphyria that is due to a deficiency of HYDROXYMETHYLBILANE SYNTHASE in the LIVER, the third enzyme in the 8-enzyme biosynthetic pathway of HEME. Clinical features are recurrent and life-threatening neurologic disturbances, ABDOMINAL PAIN, and elevated level of AMINOLEVULINIC ACID and PORPHOBILINOGEN in the urine.
Porphyrias
A diverse group of metabolic diseases characterized by errors in the biosynthetic pathway of HEME in the LIVER, the BONE MARROW, or both. They are classified by the deficiency of specific enzymes, the tissue site of enzyme defect, or the clinical features that include neurological (acute) or cutaneous (skin lesions). Porphyrias can be hereditary or acquired as a result of toxicity to the hepatic or erythropoietic marrow tissues.
Protoporphyria, Erythropoietic
An autosomal dominant porphyria that is due to a deficiency of FERROCHELATASE (heme synthetase) in both the LIVER and the BONE MARROW, the last enzyme in the 8-enzyme biosynthetic pathway of HEME. Clinical features include mainly neurological symptoms, rarely cutaneous lesions, and elevated levels of protoporphyrin and COPROPORPHYRINS in the feces.
About the E80 Code Range
These disorders affect how the body processes porphyrin or bilirubin. The category also has a subdivision for defects of catalase and peroxidase.
Porphyria is divided into hereditary erythropoietic porphyria, porphyria cutanea tarda, and other or unspecified porphyria. E80.2 splits further into unspecified, acute intermittent (hepatic), and other porphyria. E80.3 identifies catalase and peroxidase defects. The bilirubin subdivisions name Gilbert syndrome and Crigler-Najjar syndrome separately, followed by other and unspecified disorders of bilirubin metabolism.
Questions About This Page
How many billable codes are in the E80 range?
Of the 12 codes in this range, 10 are billable and valid for claim submission from October 1, 2026 through September 30, 2027. Category header codes group them but cannot be reported on claims.
What does the E80 range classify?
The range classifies disorders of porphyrin and bilirubin metabolism. Each code links to its own reference page with billing status, MS-DRG grouping, coding notes, and clinical information.
Related References
Source: CMS FY 2027 ICD-10-CM Tabular List and order file, effective October 1, 2026 through September 30, 2027.
