D81.81
Non-Billable

Biotin-dependent carboxylase deficiency

Non-Billable / Non-Specific Code

What is Biotin-dependent carboxylase deficiency?

ICD-10-CM D81.81 is the diagnosis code for Biotin-dependent carboxylase deficiency. This code falls under the section "Certain disorders involving the immune mechanism" within Chapter 3 — Diseases of the blood and blood-forming organs and certain disorders involving the immune mechanism (D50-D89). It is a non-billable/non-specific ICD-10-CM code that should not be used for reimbursement. A more specific sub-code is required for billing. Medical coders and healthcare providers use this code to document and classify diagnoses in electronic health records, insurance claims, and clinical databases.

💡 Inclusions
⛔ Excludes1

Coding Guidelines for D81.81

✅ This Code Includes

  • Multiple carboxylase deficiency

⛔ Type 1 Excludes (Never code together)

The following conditions should never be coded at the same time as D81.81. They are mutually exclusive:

  • biotin-dependent carboxylase deficiency due to dietary deficiency of biotin (E53.8)

ICD-10-CM Code Hierarchy

Understanding where D81.81 sits in the ICD-10-CM classification helps ensure proper coding:

Sub-Codes 3 entries
D81.810Biotinidase deficiencyBillableD81.818Other biotin-dependent carboxylase deficiencyBillableD81.819Biotin-dependent carboxylase deficiency, unspecifiedBillable
Related Codes

Frequently Asked Questions

Is D81.81 a billable ICD-10-CM code?

No, D81.81 is a non-billable/non-specific code. You should use a more specific sub-code for billing and reimbursement.

What does D81.81 mean?

D81.81 is the ICD-10-CM diagnosis code for "Biotin-dependent carboxylase deficiency". It is used by healthcare providers to classify and document this condition in medical records and insurance claims.

What is the parent code of D81.81?

The parent code of D81.81 is D81.8 ("Other combined immunodeficiencies"). D81.81 provides a more specific classification within this category.

What section is D81.81 in?

D81.81 is located in Section D80-D89 — "Certain disorders involving the immune mechanism" within Chapter 3 of the ICD-10-CM Tabular List.

What are the sub-codes of D81.81?

D81.81 has 3 sub-code(s) that provide more specific detail: D81.810, D81.818, D81.819.

When should I use D81.81?

Use D81.81 when the patients documented diagnosis matches "Biotin-dependent carboxylase deficiency" and the clinical documentation supports this level of specificity. Always verify with the latest ICD-10-CM guidelines and payer requirements.

People Also Ask

What is the ICD-10 code for Biotin-dependent carboxylase deficiency?

The ICD-10-CM code for Biotin-dependent carboxylase deficiency is D81.81.

Can D81.81 be used for primary diagnosis?

No, D81.81 is non-billable. Use a more specific sub-code for primary diagnosis billing.

What chapter is D81.81 in?

D81.81 is in Chapter 3 of the ICD-10-CM Tabular List.

What conditions are excluded from D81.81?

Type 1 Excludes for D81.81 include: biotin-dependent carboxylase deficiency due to dietary deficiency of biotin (E53.8).

Is D81.81 valid for 2026?

Yes, D81.81 is a valid ICD-10-CM code for the 2026 fiscal year, subject to official CMS updates.

See Also

← PreviousD81.8Other combined immunodeficienciesNext →D81.810Biotinidase deficiency
Code Details
D81.81
Biotin-dependent carboxylase deficiency
Non-Billable / Non-Specific Code
3 — Diseases of the blood and blood-forming organs and certain disorders involving the immune mechanism (D50-D89)
D80-D89 — Certain disorders involving the immune mechanism
2026 ICD-10-CM