ICD-10 Code D81.818 – Other biotin-dependent carboxylase deficiency (2026): Diagnosis, Symptoms & Billing Guide
2026 ICD-10-CM Diagnosis Code D81.818 – Other biotin-dependent carboxylase deficiency
What it is
D81.818 identifies a disorder caused by deficiency of biotin-dependent carboxylase activity. It is used when documentation specifies this condition and does not name a more specific inherited metabolic diagnosis.
Clinical signs
Clinical features vary; refer to documentation. Standard references describe metabolic abnormalities, neurologic findings, and possible skin or hair changes in biotin-related enzyme deficiency states.
When to use this code
Use this code when the record clearly documents other biotin-dependent carboxylase deficiency. It may apply during genetic, metabolic, or pediatric evaluations when the provider identifies the deficiency but does not specify a different ICD-10 option. Check documentation if the chart only says “biotin deficiency” or another nonspecific metabolic disorder.
Do not use for
Do not use this code for simple dietary biotin deficiency or for unrelated vitamin deficiency diagnoses. If the documentation names a different inherited carboxylase disorder, code that specific condition instead.
Coding tip
Code from the provider’s exact wording and confirm whether the deficiency is inherited, acquired, or unspecified.