E80.29

Applicable To

• Hereditary coproporphyria

Approximate Synonyms

• Erythropoietic protoporphyria

• Protoporphyria

Clinical Information

• An autosomal dominant inherited disorder of porphyrin metabolism caused by deficiency of the enzyme coproporphyrinogen oxidase. It results in neurologic damage and can include abdominal pain, constipation and psychiatric manifestations.

• 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.

Details

• E80.29 is a billable/specific ICD-10-CM code that can be used to indicate a diagnosis for reimbursement purposes.

• The 2023 edition of ICD-10-CM E80.29 became effective on October 1, 2022.

• This is the American ICD-10-CM version of E80.29 – other international versions of ICD-10 E80.29 may differ.