ICD-10-CM 2027 diagnosis code

D81.81Biotin-dependent carboxylase deficiency

Not billable — use a more specific code

D81.81 is a header code, so it can't be reported by itself. Choose one of the 3 more specific codes beneath it.

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 D81.81

D81.81 is the ICD-10-CM diagnosis code for biotin-dependent carboxylase 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 a header code, so it can't be reported by itself; one of its 3 subcodes must be used instead.

Within D81.8 (other combined immunodeficiencies), D81.81 is specifically for biotin-dependent carboxylase deficiency. Related codes cover activated Phosphoinositide 3-kinase Delta Syndrome [APDS] (D81.82) and other combined immunodeficiencies (D81.89).

To report this condition, pick the subcode that matches the documentation: D81.810 (deficiency), D81.818 (deficiency) and D81.819 (unspecified).

Don't report D81.81 together with the conditions in its Excludes1 note: biotin-dependent carboxylase deficiency due to dietary deficiency of biotin (E53.8).

It has been part of ICD-10-CM since the code set took effect on October 1, 2015 and hasn't changed since.

Specific codes under D81.81

Coding notes

Applicable toConditions and synonyms classified to this code

Multiple carboxylase deficiency

Excludes1Not coded here — never report together with this code

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

Notes that apply from higher levels

Instructions written at a parent level also apply to D81.81.

› From D81 Combined immunodeficiencies

Excludes1

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.

Code history

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2027

FY2016 (effective 10/1/2015): Added (first year of ICD-10-CM)
No changes since FY2016.

Common questions about D81.81

Is D81.81 billable?: No. D81.81 is a header code. Report one of its more specific subcodes instead.