ICD-10 Code D81.810 – Biotinidase deficiency (2026): Diagnosis, Symptoms & Billing Guide
2026 ICD-10-CM Diagnosis Code D81.810 – Biotinidase deficiency
What it is
D81.810 identifies biotinidase deficiency, an inherited disorder that prevents the body from recycling biotin properly. This can lead to a functional biotin shortage affecting metabolism.
Clinical signs
Clinical features vary; refer to documentation. Commonly reported findings include seizures, developmental delay, hypotonia, hearing loss, vision problems, and skin rash or alopecia.
When to use this code
Use this code when the provider documents biotinidase deficiency as a confirmed diagnosis, whether detected on newborn screening or later in childhood. You may also use it for follow-up visits for the established disorder. Do not code based on suspicion alone.
Do not use for
Do not use this code for simple biotin deficiency, other inborn errors of metabolism, or nonspecific nutritional problems. Check documentation if the record only says “low biotin” without confirmed biotinidase deficiency.
Coding tip
Confirm that the chart names biotinidase deficiency specifically; if it only mentions abnormal screening, you need documented confirmation before coding.