ICD-10-CM 2027
ICD-10 code for acute intermittent porphyria
From the official ICD-10-CM alphabetic index entry “Porphyria (South African) › acute intermittent (hepatic) (Swedish)”. Page updated September 29, 2026.
About coding acute intermittent porphyria
The ICD-10-CM code for acute intermittent porphyria is E80.21 (Acute intermittent (hepatic) porphyria).
Within E80.2 (other and unspecified porphyria), E80.21 is specifically for acute intermittent (hepatic) porphyria. Related codes cover unspecified porphyria (E80.20) and other porphyria (E80.29).
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 acute intermittent porphyria ICD-10 codes
What is the ICD-10 code for acute intermittent porphyria?
- E80.21 — Acute intermittent (hepatic) porphyria.
Is E80.21 billable?
- Yes. E80.21 is a billable/specific ICD-10-CM code valid for FY2027 (October 1, 2026 – September 30, 2027).
Can E80.21 be used as a principal diagnosis?
- Yes. The Medicare Code Editor doesn't restrict E80.21 as a principal diagnosis.
Is E80.21 a CC or MCC?
- E80.21 is a CC (complication or comorbidity) under MS-DRG v44.0, unless excluded by the principal diagnosis.