ICD-10 Code E80.21 – Acute intermittent (hepatic) porphyria (2026): Diagnosis, Symptoms & Billing Guide
2026 ICD-10-CM Diagnosis Code E80.21 – Acute intermittent (hepatic) porphyria
What it is
E80.21 identifies acute intermittent (hepatic) porphyria, an inherited disorder of heme synthesis that affects the liver. Use it when documentation names this specific acute porphyria.
Clinical signs
Clinical features vary; refer to documentation. Typical records mention recurrent abdominal pain, nausea, constipation, neuropathic symptoms, or psychiatric changes during acute attacks.
When to use this code
Use E80.21 when the provider documents acute intermittent porphyria, acute hepatic porphyria, or AIP. It is appropriate for confirmed diagnoses, active disease, or encounters for evaluation of a documented history of this condition. If the note is nonspecific, check documentation before coding.
Do not use for
Do not use this code for other porphyrias, such as cutaneous or unspecified types, unless the record clearly states acute intermittent porphyria. Do not assign it when porphyria is only suspected or ruled out.
Coding tip
Code only the exact porphyria documented; if the subtype is unclear, query the provider.