ICDcodes.org
FY2027

D81.81Biotin-dependent carboxylase deficiency

Not billable

Is D81.81 billable?

No — D81.81 is a non-billable header code. D81.81 groups more specific codes and cannot be submitted on a claim. A valid ICD-10-CM code must be reported at the highest level of specificity available in its branch. Select one of the 3 billable codes beneath D81.81.

D81.81 at a glance

CodeD81.81
DescriptionBiotin-dependent carboxylase deficiency
BillableNo — header code
Code setICD-10-CM FY2027
Valid for encountersOctober 1, 2026 – September 30, 2027

Excludes1 — never code together

The conditions below can never be reported together with D81.81 on the same claim. An Excludes1 note means the two conditions cannot occur in the same patient, so reporting both is a coding error.

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

Inclusion terms

Alternative wording in documentation that is classified to D81.81.

  • Multiple carboxylase deficiency

Excludes1 — never code together — inherited from D80-D89

The conditions below can never be reported together with D81.81 on the same claim. An Excludes1 note means the two conditions cannot occur in the same patient, so reporting both is a coding error.

  • autoimmune disease (systemic) NOS (M35.9)
  • functional disorders of polymorphonuclear neutrophils (D71-)

Excludes1 — never code together — inherited from D81

The conditions below can never be reported together with D81.81 on the same claim. An Excludes1 note means the two conditions cannot occur in the same patient, so reporting both is a coding error.

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

Excludes2 — not included here — inherited from Chapter 3

The conditions below are not part of D81.81, but a patient may have both at the same time. When documentation supports it, D81.81 and the excluded code may both be reported.

  • autoimmune disease (systemic) NOS (M35.9)
  • certain conditions originating in the perinatal period (P00-P96)
  • complications of pregnancy, childbirth and the puerperium (O00-O9A)
  • congenital malformations, deformations and chromosomal abnormalities (Q00-Q99)
  • endocrine, nutritional and metabolic diseases (E00-E88)
  • human immunodeficiency virus [HIV] disease (B20)
  • injury, poisoning and certain other consequences of external causes (S00-T88)
  • neoplasms (C00-D49)
  • symptoms, signs and abnormal clinical and laboratory findings, not elsewhere classified (R00-R94)

Excludes2 — not included here — inherited from D80-D89

The conditions below are not part of D81.81, but a patient may have both at the same time. When documentation supports it, D81.81 and the excluded code may both be reported.

  • human immunodeficiency virus [HIV] disease (B20)

What codes fall under D81.81? (3)

How long has D81.81 existed?

D81.81 has been in ICD-10-CM since at least FY2016, the earliest fiscal year on record here.

Derived from the official order files for FY2016–FY2026. FY2017 and FY2020 publish no order file, so those years are not covered.

What other codes are in the D81.8 family? (2)