Is D81.81 a billable ICD-10-CM code?

No. D81.81 is a non-billable header code. Code to a higher level of specificity using one of its child codes.

Where does D81.81 sit in the tabular list?

Diseases of the blood and blood-forming organs and certain disorders involving the immune mechanism (D50-D89), in the block Certain disorders involving the immune mechanism (D80-D89).

What cannot be coded together with D81.81?

D81.81 carries Excludes1 notes, which means the conditions they list can never be coded together with it on the same encounter. Some are written on the categories above D81.81 (D81) and apply to it just the same.

D81.81

D81.81 — Biotin-dependent carboxylase deficiency

Non-billable headerICD-10-CM

D81.81 is a non-billable header ICD-10-CM code for biotin-dependent carboxylase deficiency. It cannot be billed on its own: choose one of the more specific child codes below.

Excludes1 — never code together

These conditions cannot occur together. Billing both on the same encounter is a denial.

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

Inclusion terms

Alternative wordings a physician may use in the chart for this same code.

Multiple carboxylase deficiency

Excludes1 inherited from the categories above D81.81

These are not written on D81.81, but they bind it: a note on a category applies to every code beneath it. Billing D81.81 with anything listed here is a denial.

Written on D81 — Combined immunodeficiencies

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

Other codes that can never be billed with D81.81

These codes carry an Excludes1 note pointing at D81.81. The conflict binds both ways, but ICD-10-CM only writes it down on one side — so you would not find this by reading D81.81 alone.

E79Disorders of purine and pyrimidine metabolism

Risk adjustment

D81.81 does not map to an HCC and does not risk-adjust under CMS-HCC V28. That is normal — most ICD-10 codes do not. It still needs to be coded correctly; it just will not move a RAF score.

Code history

D81.81 has not changed since FY2024 — no addition, revision or deletion across the three most recent ICD-10-CM releases.

Code to one of these instead D81.81

3 child codes. Green means billable.

Related codes at this level

Codes that share D81.8. If D81.81 is not quite right, the correct code is usually one of these.

Questions about D81.81

Is D81.81 a billable ICD-10-CM code?: No. D81.81 is a non-billable header code. Code to a higher level of specificity using one of its child codes.
Where does D81.81 sit in the tabular list?: Diseases of the blood and blood-forming organs and certain disorders involving the immune mechanism (D50-D89), in the block Certain disorders involving the immune mechanism (D80-D89).
What cannot be coded together with D81.81?: D81.81 carries Excludes1 notes, which means the conditions they list can never be coded together with it on the same encounter. Some are written on the categories above D81.81 (D81) and apply to it just the same.