2027 ICD-10-CM Diagnosis Code D81.818Other biotin-dependent carboxylase deficiency

ICD-10-CM Codes›D50–D89›D80-D89›D81

ICD-10-CM D81.818
CMSSource: CMS FY 2027 ICD-10-CM dataset · Effective Oct 1, 2026 – Sep 30, 2027

D81.818 is a billable ICD-10-CM diagnosis code for other biotin-dependent carboxylase deficiency. It is valid on HIPAA claims for fiscal year 2027 (October 1, 2026 through September 30, 2027) and groups to MS-DRG 640 through 641. Coders also document this condition as holocarboxylase synthase deficiency. In AHRQ's Clinical Classifications Software (CCSR), this diagnosis falls under Immunity disorders and Other specified and unspecified nutritional and metabolic disorders.

Code Identity

ICD-10-CM Code
D81.818
Billable Status
Yes — Valid for Submission
Code Describes
Other biotin-dependent carboxylase deficiency
Short Description
Other biotin-dependent carboxylase deficiency
Same as the full description in the CMS dataset.
Parent Code
D81.81
Biotin-dependent carboxylase deficiency

Code Classification

ChapterD50–D89Diseases of the blood and blood-forming organs and certain disorders involving the immune mechanism
SectionD80-D89Certain disorders involving the immune mechanism
CategoryD81Combined immunodeficiencies
This CodeD81.818Other biotin-dependent carboxylase deficiency

Approximate SynonymsGuidance

Alternate terms and clinical phrases that map to this code.

  • Holocarboxylase synthase deficiency
  • Multiple carboxylase deficiency

Instructional NotesGuidance

Instructions from the official ICD-10-CM Tabular List that apply to D81.818: its own notes plus those printed at D81.81, D81, block D80-D89, and Chapter 3. A note printed at a category, block or chapter applies to every code under it.

Includes

Further defines, or gives examples of, the content of the category.

From Block D80-D89 Certain disorders involving the immune mechanism applies to 83 codes
  • defects in the complement system
  • immunodeficiency disorders, except human immunodeficiency virus [HIV] disease
  • sarcoidosis

Applicable To

Conditions this code is used for: synonyms of the title or, for "other specified" codes, the conditions assigned to it. The list is not exhaustive.

  • Holocarboxylase synthetase deficiency
  • Other multiple carboxylase deficiency

Excludes1

Not coded here: the excluded code is never reported together with this one, unless the two conditions are unrelated.

From D81.81 Biotin-dependent carboxylase deficiency applies to 3 codes
  • biotin-dependent carboxylase deficiency due to dietary deficiency of biotin (E53.8)
From D81 Combined immunodeficiencies applies to 17 codes
  • autosomal recessive agammaglobulinemia (Swiss type) (D80.0)
From Block D80-D89 Certain disorders involving the immune mechanism applies to 83 codes
  • autoimmune disease (systemic) NOS (M35.9)
  • functional disorders of polymorphonuclear neutrophils (D71-)

Excludes2

Not included here: the excluded condition is not part of this code, but a patient may have both, so both codes may be reported.

From Block D80-D89 Certain disorders involving the immune mechanism applies to 83 codes
  • human immunodeficiency virus [HIV] disease (B20)
From Chapter 3 (D50-D89) Diseases of the blood and blood-forming organs and certain disorders involving the immune mechanism applies to 324 codes
  • autoimmune disease (systemic) NOS (M35.9)
  • certain conditions originating in the perinatal period (P00-P96)
  • complications of pregnancy, childbirth and the puerperium (O00-O9A)
  • congenital malformations, deformations and chromosomal abnormalities (Q00-Q99)
  • endocrine, nutritional and metabolic diseases (E00-E88)
  • human immunodeficiency virus [HIV] disease (B20)
  • injury, poisoning and certain other consequences of external causes (S00-T88)
  • neoplasms (C00-D49)
  • symptoms, signs and abnormal clinical and laboratory findings, not elsewhere classified (R00-R94)

Source: CMS ICD-10-CM Tabular List. How to read instructional notes.

Referenced in Other NotesGuidance

Instructional notes printed at other codes that name D81.818, its category, or a range that includes it.

Excludes1 3

These codes carry an Excludes1 note naming D81.818: they are not reported together with it, unless the two conditions are unrelated.

  • E79 Disorders of purine and pyrimidine metabolism
    combined immunodeficiency disorders (D81.-) names D81.-, which includes this code
  • D72 Other disorders of white blood cells
    immunity disorders (D80-D89) names D80-D89, which includes this code
  • D84.81 Immunodeficiency due to conditions classified elsewhere
    certain disorders involving the immune mechanism (D80-D83, D84.0, D84.1, D84.9) names D80-D83, which includes this code

Index to Diseases and InjuriesGuidance

Alphabetical index entries that point to this code.

Clinical ClassificationClinical

AHRQ’s CCSR groups this code into broader clinical categories.

CCSR BLD008
Immunity disorders
Default principal diagnosis: inpatient Yes · outpatient Yes
CCSR END016
Other specified and unspecified nutritional and metabolic disorders
Default principal diagnosis: inpatient No · outpatient No

Patient EducationClinical

Immune System and Disorders

Your immune system is a complex network of cells, tissues, and organs. Together they help the body fight infections and other diseases.

The full article covers:

  • What is the immune system?
  • What are the parts of the immune system?
  • How does the immune system work?
  • What are the types of immunity?
  • What can go wrong with the immune system?

Read the full article at MedlinePlus

Courtesy of MedlinePlus, a service of the U.S. National Library of Medicine.

Convert D81.818 to ICD-9-CMHistory

The closest ICD-9-CM equivalents under the General Equivalence Mappings.

ICD-9-CM
266.2 B-complex defic NEC
Approximate The match is approximate rather than exact.

Code HistoryHistory

FY 2016AddedAdded to the ICD-10-CM code setEffective October 1, 2015, the first year of ICD-10-CM.
FY 2017–2026No changes
FY 2027CurrentCurrent code set, no changesEffective October 1, 2026 through September 30, 2027.

Questions About D81.818Overview

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

The ICD-10-CM code for other biotin-dependent carboxylase deficiency is D81.818 (sometimes written as D81818). It is billable on HIPAA-covered claims from October 1, 2026 through September 30, 2027.

Is D81.818 (Biotin-dependent carboxylase deficiency) a billable code?

Yes. This is a billable ICD-10-CM code, specific enough to report other biotin-dependent carboxylase deficiency on HIPAA-covered claims from October 1, 2026 through September 30, 2027.

What MS-DRG does D81.818 group to?

When other biotin-dependent carboxylase deficiency is the principal diagnosis on an inpatient stay, it groups to MS-DRG 641, 640, with relative weights from 0.7864 to 1.3392 depending on complications. Higher weights mean higher Medicare reimbursement.

Can D81.818 be reported with E53.8?

Not as a rule. The Excludes1 note at D81.81, which applies to D81.818, lists "biotin-dependent carboxylase deficiency due to dietary deficiency of biotin (E53.8)". An Excludes1 note means the excluded code is never reported together with this one; the only exception is when the two conditions are unrelated to each other (Official Guidelines, Section I.A.12.a).

What is the ICD-9 equivalent of D81.818?

Under the General Equivalence Mappings, other biotin-dependent carboxylase deficiency converts to ICD-9-CM 266.2 (b-complex defic NEC). The mapping is approximate, so confirm the match fits the documentation.