ICD-10 Code D81.81 – Biotin-dependent carboxylase deficiency (2026): Diagnosis, Symptoms & Billing Guide
2026 ICD-10-CM Diagnosis Code D81.81 – Biotin-dependent carboxylase deficiency
What it is
D81.81 identifies biotin-dependent carboxylase deficiency, a rare metabolic disorder affecting enzymes that need biotin to function. It is used when documentation confirms this specific inherited or acquired enzyme deficiency.
Clinical signs
Clinical features vary; refer to documentation. Reported findings may include metabolic decompensation, developmental delay, seizures, poor feeding, or other signs of impaired carboxylase activity.
When to use this code
Use D81.81 when the record specifically states biotin-dependent carboxylase deficiency or an equivalent confirmed diagnosis. Apply it for encounters focused on evaluating, monitoring, or managing this condition. If the note only mentions suspected metabolic disease, check documentation before coding.
Do not use for
Do not use this code for general biotin deficiency, nonspecific metabolic abnormalities, or unrelated enzyme disorders. If the provider has not confirmed this diagnosis, check documentation.
Coding tip
Code only when the clinician documents the exact deficiency; otherwise, query or wait for clearer confirmation.