ICD-10 Code E83.821 – ENPP1 deficiency causing generalized arterial calcification of infancy (2026): Diagnosis, Symptoms & Billing Guide
2026 ICD-10-CM Diagnosis Code E83.821 – ENPP1 deficiency causing generalized arterial calcification of infancy
What it is
E83.821 identifies ENPP1 deficiency, a rare inherited disorder that can cause generalized arterial calcification of infancy. Use it when documentation states this specific genetic cause.
Clinical signs
Clinical features may include early arterial calcification, vascular narrowing, and related infantile cardiovascular complications. Clinical features vary; refer to documentation.
When to use this code
Use this code when the provider documents ENPP1 deficiency as the cause of generalized arterial calcification of infancy. It may also apply when the record confirms the genetic diagnosis and links it to the arterial calcification disorder. Check documentation if the chart only notes calcification without a stated cause.
Do not use for
Do not use this code for nonspecific vascular calcification or other metabolic disorders without ENPP1 deficiency. Do not assign it when the diagnosis is only suspected or not confirmed.
Coding tip
Code the documented genetic diagnosis, not just the imaging finding, and verify the causal link in the provider note.