ICD-10-CM 2027
ICD-10 code for erythropoietic porphyria
From the official ICD-10-CM alphabetic index entry “Porphyria (South African) › erythropoietic (congenital) (hereditary)”. Page updated September 29, 2026.
About coding erythropoietic porphyria
The ICD-10-CM code for erythropoietic porphyria is E80.0 (Hereditary erythropoietic porphyria).
Within E80 (disorders of porphyrin and bilirubin metabolism), E80.0 is specifically for hereditary erythropoietic porphyria. Related codes cover porphyria cutanea tarda (E80.1), other and unspecified porphyria (E80.2), defects of catalase and peroxidase (E80.3), Gilbert syndrome (E80.4), Crigler-Najjar syndrome (E80.5), other disorders of bilirubin metabolism (E80.6) and disorder of bilirubin metabolism, unspecified (E80.7).
As a secondary diagnosis it is a CC (complication or comorbidity), which can move an inpatient stay into the "with CC" MS-DRG of its family, unless the principal diagnosis excludes it.
In MS-DRG v44.0, it is part of the grouping logic for DRG 642 (Inborn and Other Disorders of Metabolism, relative weight 1.3219), in MDC 10 (Endocrine, Nutritional and Metabolic Diseases and Disorders), as a principal or secondary diagnosis. The DRG a claim lands in depends on the principal diagnosis, procedures and any CC/MCC secondary diagnoses.
Common questions about erythropoietic porphyria ICD-10 codes
What is the ICD-10 code for erythropoietic porphyria?
- E80.0 — Hereditary erythropoietic porphyria.
Is E80.0 billable?
- Yes. E80.0 is a billable/specific ICD-10-CM code valid for FY2027 (October 1, 2026 – September 30, 2027).
Can E80.0 be used as a principal diagnosis?
- Yes. The Medicare Code Editor doesn't restrict E80.0 as a principal diagnosis.
Is E80.0 a CC or MCC?
- E80.0 is a CC (complication or comorbidity) under MS-DRG v44.0, unless excluded by the principal diagnosis.