D81.81 – Biotin-dependent carboxylase deficiency
ICD-10-CM 2027 diagnosis code · Certain disorders involving the immune mechanism
ICD-10-CM code
D81.81
- Code
D81.81(claims format:D8181)- Description
- Biotin-dependent carboxylase deficiency
- Billable
- No – use a more specific code below
- 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
Billable codes under D81.81
D81.81 is a header code and cannot be used on claims. Choose the most specific code:
Notes for D81.81
Applicable to:
- Multiple carboxylase deficiency
Excludes1 (not coded here):
- biotin-dependent carboxylase deficiency due to dietary deficiency of biotin (E53.8)
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)
Block D80-D89
Includes:
- defects in the complement system
- immunodeficiency disorders, except human immunodeficiency virus [HIV] disease
- sarcoidosis
Excludes1 (not coded here):
Excludes2 (not included here):
- human immunodeficiency virus [HIV] disease (B20)
Related codes in D81.8
Frequently asked questions
What is ICD-10 code D81.81?
D81.81 is the ICD-10-CM code for biotin-dependent carboxylase deficiency, in the block D80-D89 (Certain disorders involving the immune mechanism).
Is D81.81 a billable code?
No. D81.81 is a header code with more specific child codes; report one of the billable codes listed above.
What category does D81.81 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.