D81.81 Biotin-dependent carboxylase deficiency

⚠ Non-Billable ICD-10-CM 2026
Open D81.81 in Web App →

The ICD-10-CM code for Biotin-dependent carboxylase deficiency is D81.81 (FY2026). It is a non-billable category code — select a more specific code below for claims.

Classification

Section
D80-D89: Certain disorders involving the immune mechanism (D80-D89)
Category D81
21 codes (17 billable)
FY2026 Status
Stable since FY2024
Excludes1
Not coded here. These conditions should NOT be coded together with D81.81.
  • biotin-dependent carboxylase deficiency due to dietary deficiency of biotin (E53.8)

Inclusion Terms

  • Multiple carboxylase deficiency

Official Coding Guidelines

Chapter 3 TOC entry

3. Chapter 3: Disease of the blood and blood-forming organs and certain disorders involving the immune mechanism (D50-D89) ............................................................................................. 39

— ICD-10-CM Official Guidelines for Coding and Reporting, FY2026, Section I.C.3
Reserved for future guideline expansion

Reserved for future guideline expansion ...................................................................................... 39

— ICD-10-CM Official Guidelines for Coding and Reporting, FY2026, Section I.C.3.Reserved

Source: CMS — ICD-10-CM Official Guidelines for Coding and Reporting, FY2026

References

Related Codes

Parent Code

D81 Combined immunodeficiencies

More Specific Codes

D81.810 Biotinidase deficiency D81.818 Other biotin-dependent carboxylase deficiency D81.819 Biotin-dependent carboxylase deficiency, unspecified

Related Codes in D81

D81 Combined immunodeficiencies D81.0 Severe combined immunodeficiency [SCID] with reticular dysgenesis D81.1 Severe combined immunodeficiency [SCID] with low T- and B-cell numbers D81.2 Severe combined immunodeficiency [SCID] with low or normal B-cell numbers D81.3 Adenosine deaminase [ADA] deficiency D81.30 Adenosine deaminase deficiency, unspecified D81.31 Severe combined immunodeficiency due to adenosine deaminase deficiency D81.32 Adenosine deaminase 2 deficiency D81.39 Other adenosine deaminase deficiency D81.4 Nezelof's syndrome D81.5 Purine nucleoside phosphorylase [PNP] deficiency D81.6 Major histocompatibility complex class I deficiency D81.7 Major histocompatibility complex class II deficiency D81.8 Other combined immunodeficiencies D81.810 Biotinidase deficiency D81.818 Other biotin-dependent carboxylase deficiency D81.819 Biotin-dependent carboxylase deficiency, unspecified D81.82 Activated Phosphoinositide 3-kinase Delta Syndrome [APDS] D81.89 Other combined immunodeficiencies D81.9 Combined immunodeficiency, unspecified
Search all 70,000+ ICD-10-CM codes →
Reviewed by Prajwal Shrestha, CPC, CRC
Certified Professional Coder (CPC) and Certified Risk Adjustment Coder (CRC) · AAPC Member ID 01997614 · About · Editorial policy · Content last reviewed: 2025-10-01

Get HCC Mappings, Clinical Summaries, and More

Access premium features including HCC risk adjustment, FDA drug indications, AI coding assistant, and comprehensive clinical context.

Start Free Trial