E80.0 – Hereditary erythropoietic porphyria

ICD-10-CM 2027 diagnosis code · Metabolic disorders

Billable code

ICD-10-CM code E80.0

Code: E80.0 (claims format: E800)
Description: Hereditary erythropoietic porphyria
Billable: Yes – valid for HIPAA-covered transactions
Valid for: Dates of service October 1, 2026 – September 30, 2027 (FY2027)
Chapter: 4. Endocrine, nutritional and metabolic diseases (E00-E89)
Block: E70-E88 Metabolic disorders
Parent codes: E80

Notes for E80.0

Applicable to:

Congenital erythropoietic porphyria
Erythropoietic protoporphyria

Notes inherited from parent codes

Instructional notes at a category or block level apply to every code below it.

E80 – Disorders of porphyrin and bilirubin metabolism

Includes:

defects of catalase and peroxidase

Block E70-E88

Excludes1 (not coded here):

androgen insensitivity syndrome (E34.5-)
congenital adrenal hyperplasia (E25.0)
hemolytic anemias attributable to enzyme disorders (D55.-)
Marfan syndrome (Q87.4-)
5-alpha-reductase deficiency (E29.1)

Excludes2 (not included here):

Ehlers-Danlos syndromes (Q79.6-)

Index terms for E80.0

Entries in the ICD-10-CM alphabetic index that lead to this code:

Related codes in E80

E80.1 – Porphyria cutanea tardaBillable
E80.2 – Other and unspecified porphyriaHeader
E80.3 – Defects of catalase and peroxidaseBillable
E80.4 – Gilbert syndromeBillable
E80.5 – Crigler-Najjar syndromeBillable
E80.6 – Other disorders of bilirubin metabolismBillable
E80.7 – Disorder of bilirubin metabolism, unspecifiedBillable

Frequently asked questions

What is ICD-10 code E80.0?

E80.0 is the ICD-10-CM code for hereditary erythropoietic porphyria, in the block E70-E88 (Metabolic disorders).

Is E80.0 a billable code?

Yes. E80.0 is a billable/specific code that can be used to indicate a diagnosis for reimbursement purposes.

What category does E80.0 belong to?

It belongs to category E80 – Disorders of porphyrin and bilirubin metabolism.

Source: CDC/NCHS ICD-10-CM FY2027 code files, tabular list and index. Reference only – code assignment must follow the official ICD-10-CM guidelines and documentation in the medical record.