D81.81
Header — not billableBiotin-dependent carboxylase deficiency
The ICD-10 code for biotin-dependent carboxylase deficiency is D81.81.
D81.81 is a non-billable header code. It groups related conditions but cannot be used on its own for reimbursement — select one of the 3 more specific codes below.
Clinical notes
Excludes1 (not coded here)
An Excludes1 note is a pure exclusion: the excluded condition and this code should never be reported together, because the two conditions cannot occur at the same time (e.g. a congenital form vs. an acquired form of the same disease).
- biotin-dependent carboxylase deficiency due to dietary deficiency of biotin (E53.8)
Also known as
Alternate wording, synonyms, or specific conditions that fall under this code, listed to help confirm you've picked the right one.
- Multiple carboxylase deficiency
Documentation support
General coding-documentation guidance — not a substitute for payer-specific requirements or professional coding judgment.
- •This is a non-billable header code. Documentation must support a more specific child code before this diagnosis can be reported on a claim.
- •Confirm the excluded condition(s) listed above are not also present — Excludes1 conditions cannot be coded together with this one.
- •As a general rule, code to the highest level of specificity supported by the documentation in the medical record.
Related codes in this category
- D81.82Activated Phosphoinositide 3-kinase Delta Syndrome [APDS]
- D81.89Other combined immunodeficiencies
- D81.81Biotin-dependent carboxylase deficiencynon-billable header