D81.819 ICD-10-CM Code: Biotin-dependent carboxylase deficiency, unspecified
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FY 2026 Apr update / Diseases of the blood and blood-forming organs and certain disorders involving the immune mechanism (D50-D89) / Certain disorders involving the immune mechanism (D80-D89)
D81.819
Billable / SpecificICD-10-CMOfficial ICD-10-CMCodebook guidanceBiotin-dependent carboxylase deficiency, unspecified
A rare genetic disorder affecting enzymes that depend on biotin (a B vitamin) for proper function, but the specific type of enzyme deficiency is not specified.
CMS-HCC V28
00
RAF 0
CMS-HCC V24
00
RAF 0
ACA/HHS
00
RAF 0
ESRD/PACE
00
RAF 0
RXHCC
00
RAF 0
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Code Book Path
Inclusion Terms
Official- Multiple carboxylase deficiency, unspecified
Excludes 2
OfficialICD-10-CM does not list Excludes 2 notes for D81.819 in this effective period.
Related Child Codes
Includes
OfficialICD-10-CM does not list Includes notes for D81.819 in this effective period.
Excludes 1
Official- biotin-dependent carboxylase deficiency due to dietary deficiency of biotin (E53.8)
Code First
OfficialICD-10-CM does not list Code First sequencing instructions for D81.819 in this effective period.
Use Additional
OfficialICD-10-CM does not list Use Additional Code instructions for D81.819 in this effective period.
Code Also
OfficialICD-10-CM does not list Code Also instructions for D81.819 in this effective period.
Buddy Documentation Tip
Last updated: FY2026 ICD-10-CM Apr update, Apr 1, 2026 through Sep 30, 2026. CMS-HCC V28 is 100% phased in for payment year 2026.
Is D81.819 an HCC code?
No. D81.819 is a billable ICD-10-CM code but does not map to any HCC category in V28, V24, ESRD, or RxHCC.
This code does not map to an HCC category in any model (V28, V24, ESRD, RxHCC).
What This Code Means
D81.819 is the ICD-10-CM diagnosis code for biotin-dependent carboxylase deficiency, unspecified. A rare genetic disorder affecting enzymes that depend on biotin (a B vitamin) for proper function, but the specific type of enzyme deficiency is not specified. D81.819 sits in the ICD-10-CM chapter for diseases of the blood and blood-forming organs and certain disorders involving the immune mechanism (d50-d89), within the section covering certain disorders involving the immune mechanism (d80-d89).
D81.819 is a billable ICD-10-CM code but does not map to a payment HCC under the CMS-HCC V28, V24, ESRD, or RxHCC risk adjustment models. It can be reported on Medicare Advantage encounter data submissions but it does not contribute to a beneficiary's RAF score and therefore does not affect risk-adjusted payments to the plan.
Use this code only when biotin-dependent carboxylase deficiency is documented but the specific type cannot be determined.
HCC Buddy maintains structured V28 and V24 mapping, RAF weights, and MEAT documentation criteria for D81.819 sourced directly from the CMS-HCC risk adjustment model files and the CMS ICD-10-CM code set.