D81.819Biotin-dependent carboxylase deficiency, unspecified

D81.819 is the ICD-10-CM diagnosis code for biotin-dependent carboxylase deficiency, unspecified. 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, choose D81.819 (unspecified) only when documentation doesn't support a more specific option: Biotinidase deficiency (D81.810) and other biotin-dependent carboxylase deficiency (D81.818).

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 640 (Miscellaneous Disorders of Nutrition, Metabolism, Fluids and Electrolytes with MCC, relative weight 1.3395) and DRG 641 (Miscellaneous Disorders of Nutrition, Metabolism, Fluids and Electrolytes without MCC, relative weight 0.7865), 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 266.2 (other B-complex deficiencies).