ICD-10 Code E80.0 – Hereditary erythropoietic porphyria (2026): Diagnosis, Symptoms & Billing Guide
2026 ICD-10-CM Diagnosis Code E80.0 – Hereditary erythropoietic porphyria
What it is
E80.0 identifies hereditary erythropoietic porphyria, an inherited disorder of heme synthesis. It causes buildup of porphyrins that can affect skin and red blood cells.
Clinical signs
Typical findings include photosensitivity, blistering or fragility of sun-exposed skin, and red or brown discoloration of urine or teeth. Clinical features vary; refer to documentation.
When to use this code
Use this code when the provider documents hereditary erythropoietic porphyria or congenital erythropoietic porphyria. Code it for confirmed diagnoses, genetic or metabolic workups, or follow-up visits where the condition remains active.
Do not assign it for nonspecific photosensitivity alone. If the record only says “porphyria” or another porphyria type, check documentation before coding.
Do not use for
Do not use E80.0 for acquired porphyria, porphyria cutanea tarda, or other specified porphyrias. Check documentation if the chart does not clearly identify the hereditary erythropoietic form.
Coding tip
Confirm the exact porphyria type in the assessment or problem list before assigning E80.0.