U.S. ICD-10-CM · FY 2027

D81.818 ICD-10-CM Code: Other biotin-dependent carboxylase deficiency

A clear reference for the official code description, hierarchy, and related coding information from the FY 2027 U.S. release.

Code definition

Official description

D81.818

Other biotin-dependent carboxylase deficiency

ICD-10-CM diagnosis code · United States clinical modification

Classification

Where D81.818 sits

  1. 1

    Chapter 3

    Diseases of the blood and blood-forming organs and certain disorders involving the immune mechanism (D50-D89)

  2. 2

    Section

    Certain disorders involving the immune mechanism (D80-D89)

  3. 3

    Category D81

    Combined immunodeficiencies

  4. 4

    Subcategory D81.81

    Biotin-dependent carboxylase deficiency

  5. 5

    Code D81.818

    Other biotin-dependent carboxylase deficiency

Documentation details

Notes for this code

Inclusion terms

  • Holocarboxylase synthetase deficiency
  • Other multiple carboxylase deficiency

Code first

No “code first” instruction is listed for D81.818.

Use additional code

No “use additional code” instruction is listed for D81.818.

Excludes1

Conditions that should not be reported together with this code.

No Excludes1 note is listed for D81.818.

Excludes2

Conditions not included here that may be reported separately when present.

No Excludes2 note is listed for D81.818.

Transparency

Source and release

Data release

U.S. ICD-10-CM · FY 2027

Effective date

Oct 1, 2026

This reference is generated from Vero’s ICD-10-CM description, hierarchy, and code-note dataset. The visible content and structured data share that source.

Review the CDC/NCHS ICD-10-CM source

Code-specific guidance

Frequently asked questions

What does ICD-10-CM code D81.818 mean?

D81.818 is the FY 2027 U.S. ICD-10-CM code for “Other biotin-dependent carboxylase deficiency.”

Is D81.818 a billable or specific ICD-10-CM code?

Yes. In the FY 2027 reference data used on this page, D81.818 has no child codes and is presented as a billable/specific code. Always confirm the active code set and payer requirements before use.

Where is D81.818 located in ICD-10-CM?

D81.818 is listed in Chapter 3, Diseases of the blood and blood-forming organs and certain disorders involving the immune mechanism (D50-D89), within Certain disorders involving the immune mechanism (D80-D89) (D80–D89), under category D81, Combined immunodeficiencies and subcategory D81.81, Biotin-dependent carboxylase deficiency.

What coding notes are listed for D81.818?

Its listed inclusion terms are Holocarboxylase synthetase deficiency; Other multiple carboxylase deficiency. No code-first instruction is listed. No Excludes1 note is listed. No Excludes2 note is listed.

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