ICD-10-CM 2027 diagnosis code
D81.810Biotinidase deficiency
D81.810 is a valid, billable ICD-10-CM code for biotinidase deficiency. It can be reported on claims for dates of service from October 1, 2026 – September 30, 2027.
Code unchanged since ICD-10-CM took effect on October 1, 2015. Source: official FY2027 ICD-10-CM release from CDC/NCHS and CMS. Page updated September 29, 2026. About our data
About D81.810
D81.810 is the ICD-10-CM diagnosis code for Biotinidase deficiency. It belongs to category D81 (combined immunodeficiencies), block D80-D89 (certain disorders involving the immune mechanism) and chapter 3 (diseases of the blood and blood-forming organs and certain disorders involving the immune mechanism). It is billable, so it can be reported on its own for dates of service from October 1, 2026 through September 30, 2027.
Within D81.81 (biotin-dependent carboxylase deficiency), D81.810 is specifically for Biotinidase deficiency. Related codes cover other biotin-dependent carboxylase deficiency (D81.818) and unspecified (D81.819).
It is not a CC or MCC, so as a secondary diagnosis it doesn't change the MS-DRG severity level.
In MS-DRG v44.0, it is part of the grouping logic for DRG 642 (Inborn and Other Disorders of Metabolism, relative weight 1.3219), in MDC 10 (Endocrine, Nutritional and Metabolic Diseases and Disorders), as a principal or secondary diagnosis. The DRG a claim lands in depends on the principal diagnosis, procedures and any CC/MCC secondary diagnoses.
It has been part of ICD-10-CM since the code set took effect on October 1, 2015 and hasn't changed since.
Before ICD-10, this condition was coded in ICD-9-CM as 277.6 (other deficiencies of circulating enzymes).
Notes that apply from higher levels
Instructions written at a parent level also apply to D81.810.
› From D81.81 Biotin-dependent carboxylase deficiency
- biotin-dependent carboxylase deficiency due to dietary deficiency of biotin (E53.8)
› From D81 Combined immunodeficiencies
- autosomal recessive agammaglobulinemia (Swiss type) (D80.0)
Broader instructions also apply from D80-D89 Certain disorders involving the immune mechanism and Chapter 3: Diseases of the blood and blood-forming organs and certain disorders involving the immune mechanism.
Alphabetic index entries
2 entriesTerms in the official ICD-10-CM index that lead to D81.810.
MS-DRG v44.0 grouping
All DRGs →Inpatient MS-DRGs this diagnosis can group to, as the principal or a secondary diagnosis, depending on the rest of the claim.
MDC 10 · Endocrine, Nutritional and Metabolic Diseases and Disorders
ICD-9-CM equivalent
Converter →- 277.6Other deficiencies of circulating enzymesapproximate
From the CMS 2018 General Equivalence Mappings (GEMs), the final GEMs release.
Code history
- 2016
- 2017
- 2018
- 2019
- 2020
- 2021
- 2022
- 2023
- 2024
- 2025
- 2026
- 2027
- FY2016 (effective 10/1/2015): Added (first year of ICD-10-CM)
- No changes since FY2016.
Common questions about D81.810
Is D81.810 billable?
- Yes. D81.810 is a billable/specific ICD-10-CM code valid for FY2027 (October 1, 2026 – September 30, 2027).
Can D81.810 be used as a principal diagnosis?
- Yes. The Medicare Code Editor doesn't restrict D81.810 as a principal diagnosis.
Is D81.810 a CC or MCC?
- No. D81.810 is neither a CC nor an MCC under MS-DRG v44.0.
What DRG does D81.810 group to?
- D81.810 is used in the MS-DRG v44.0 logic for MS-DRG 642 (Inborn and Other Disorders of Metabolism). Which one applies depends on whether it is the principal diagnosis and on the rest of the claim.
What is the ICD-9 code for D81.810?
- The CMS General Equivalence Mappings map D81.810 to ICD-9-CM 277.6.