2026 ICD-10-CM Diagnosis Code E80.1Porphyria cutanea tarda

ICD-10-CM Codes›E00–E89›E70-E88›E80

ICD-10-CM E80.1
CMSSource: CMS FY 2026 ICD-10-CM dataset · Effective Oct 1, 2025 – Sep 30, 2026

E80.1 is a billable ICD-10-CM diagnosis code for porphyria cutanea tarda. 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
E80.1
Billable Status
Yes — Valid for Submission
Code Describes
Porphyria cutanea tarda
Short Description
Porphyria cutanea tarda
Same as the full description in the CMS dataset.
Parent Code
E80
Disorders of porphyrin and bilirubin metabolism

Code Classification

ChapterE00–E89Endocrine, nutritional and metabolic diseases
SectionE70-E88Metabolic disorders
CategoryE80Disorders of porphyrin and bilirubin metabolism
This CodeE80.1Porphyria cutanea tarda

Approximate SynonymsGuidance

Alternate terms and clinical phrases that map to this code.

  • Acquired porphyria cutanea tarda
  • Familial porphyria cutanea tarda
  • Hepatic porphyria
  • Homozygous porphyria cutanea tarda
  • Porphyria cutanea tarda
  • Sporadic porphyria cutanea tarda
  • Uroporphyrinogen decarboxylase deficiency

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.

    • Porphyria(South African)
      • cutanea tarda (hereditary) (symptomatic)
    • Porphyria(South African)
      • hepatocutaneous type

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

Clinical InformationClinical

  • 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 Cutanea Tarda

    a cutaneous form of the genetic photosensitive disease, porphyria, that is characterized by onset in adult life and the presence of scarring bullae, hyperpigmentation, facial hypertrichosis, and sometimes sclerodermatous thickenings and alopecia. uroporphyrins are found in the urine due to a deficiency of uroporphyrinogen decarboxylase, an enzyme required for the synthesis of heme.

Patient EducationClinical

Porphyria

Porphyrias are a group of genetic disorders caused by problems with how your body makes a substance called heme. Heme is found throughout the body, especially in your blood and bone marrow, where it carries oxygen.

Read the full article at MedlinePlus

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

Convert E80.1 to ICD-9-CMHistory

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

ICD-9-CM
277.1 Dis porphyrin 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 E80.1Overview

Is E80.1 (Disorders of porphyrin and bilirubin metabolism) a billable code?

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

What is the ICD-9 equivalent of E80.1?

Under the General Equivalence Mappings, porphyria cutanea tarda converts to ICD-9-CM 277.1 (dis porphyrin 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.