D81.818 – Other biotin-dependent carboxylase deficiency

ICD-10-CM 2027 diagnosis code · Certain disorders involving the immune mechanism

Billable code

ICD-10-CM code D81.818

Code: D81.818 (claims format: D81818)
Description: Other biotin-dependent carboxylase deficiency
Billable: Yes – valid for HIPAA-covered transactions
Valid for: Dates of service October 1, 2026 – September 30, 2027 (FY2027)
Chapter: 3. Diseases of the blood and blood-forming organs and certain disorders involving the immune mechanism (D50-D89)
Block: D80-D89 Certain disorders involving the immune mechanism
Parent codes: D81 › D81.8 › D81.81

Notes for D81.818

Applicable to:

Holocarboxylase synthetase deficiency
Other multiple carboxylase deficiency

Notes inherited from parent codes

Instructional notes at a category or block level apply to every code below it.

D81 – Combined immunodeficiencies

Excludes1 (not coded here):

autosomal recessive agammaglobulinemia (Swiss type) (D80.0)

D81.81 – Biotin-dependent carboxylase deficiency

Applicable to:

Multiple carboxylase deficiency

Excludes1 (not coded here):

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

Block D80-D89

Includes:

defects in the complement system
immunodeficiency disorders, except human immunodeficiency virus [HIV] disease
sarcoidosis

Excludes1 (not coded here):

autoimmune disease (systemic) NOS (M35.9)
functional disorders of polymorphonuclear neutrophils (D71-)

Excludes2 (not included here):

human immunodeficiency virus [HIV] disease (B20)

Index terms for D81.818

Entries in the ICD-10-CM alphabetic index that lead to this code:

Related codes in D81.81

Frequently asked questions

What is ICD-10 code D81.818?

D81.818 is the ICD-10-CM code for other biotin-dependent carboxylase deficiency, in the block D80-D89 (Certain disorders involving the immune mechanism).

Is D81.818 a billable code?

Yes. D81.818 is a billable/specific code that can be used to indicate a diagnosis for reimbursement purposes.

What category does D81.818 belong to?

It belongs to category D81 – Combined immunodeficiencies.

Source: CDC/NCHS ICD-10-CM FY2027 code files, tabular list and index. Reference only – code assignment must follow the official ICD-10-CM guidelines and documentation in the medical record.