ICD10BillableValid for clinical use
ICD-10 Code E80.0: Hereditary erythropoietic porphyria
Reviewed by HealthAssure Clinical TeamUpdated 26 May 2026
What is this code?
ICD-10 codes are diagnosis classification codes used in healthcare records, reporting, coding workflows, and billing support. This code sits within the broader ICD-10 area for Endocrine, nutritional and metabolic diseases (E00-E89).
When is it used?
- May be used when a clinician documents hereditary erythropoietic porphyria in a patient's medical record.
- May appear in hospital records, claims, referrals, and clinical documentation.
- This code can be used as a clinically usable diagnosis entry in standardized coding workflows.
What it does not mean
- A code alone does not explain severity, treatment plan, or outcome.
- A medical code should not be treated as a substitute for a doctor's diagnosis or advice.
Code hierarchy
chapter
4Endocrine, nutritional and metabolic diseases (E00-E89)
block
E70-E88Metabolic disorders
category
E80Disorders of porphyrin and bilirubin metabolism
currentE80
Official coding notes
Inclusion terms
- Congenital erythropoietic porphyria
- Erythropoietic protoporphyria
Where you may see this code
You may see this code in hospital records, discharge summaries, insurance claims, encounter documentation, referrals, or other healthcare billing and coding records.
Related specialists
EndocrinologistGeneral Physician
Related codes
Sibling codes
Mapped diagnoses and classifications
SNOMED_CT 22935002high
Congenital erythropoietic porphyria
Source: SNOMED International ICD-10 map
SNOMED_CT 67312003high
Erythropoietic porphyria
Source: SNOMED International ICD-10 map
SNOMED_CT 51022005high
Erythropoietic protoporphyria
Source: SNOMED International ICD-10 map
SNOMED_CT 860859009high
Erythropoietic protoporphyria due to ferrochelatase deficiency
Source: SNOMED International ICD-10 map
SNOMED_CT 1186810009high
Erythropoietic protoporphyria due to gain of function of erythroid-specific aminolevulinic acid synthase 2
Source: SNOMED International ICD-10 map
SNOMED_CT 238053006high
Homozygous erythropoietic protoporphyria
Source: SNOMED International ICD-10 map
Coding guidelines
Compatibility
Legacy and official ICD code match exactly.
Legacy codes
E80.0
Common synonyms
Hereditary erythropoietic porphyria
Frequently asked questions
About this content
This page is prepared by HealthAssure's clinical team using official coding standards from ICD-10. AI tools assist with drafting explanations, which are then reviewed and verified by healthcare professionals for accuracy. This content is for informational purposes and does not replace professional medical advice. Meet our team.