D81.81
Biotin-dependent carboxylase deficiency
D81.81 is a non-billable ICD-10-CM code header for biotin-dependent carboxylase deficiency. Report a more specific code from it instead. It is part of the FY2027 code set, effective October 1, 2026. The more specific codes under it are D81.810 Biotinidase deficiency; D81.818 Other biotin-dependent carboxylase deficiency and D81.819 Biotin-dependent carboxylase deficiency, unspecified. Its coding notes include 4 Excludes1 entries and 10 Excludes2 entries.
Excludes1
Not coded here. The listed conditions generally should not be reported together.
- biotin-dependent carboxylase deficiency due to dietary deficiency of biotin (E53.8)
Excludes2
Not included here. Both codes may be reported if the patient has both conditions.
Also from chapter/block level (10 notes)
- human immunodeficiency virus [HIV] disease (B20)
- 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)
Includes
Further defines or gives examples of the content of the category.
Also from chapter/block level (3 notes)
- defects in the complement system
- immunodeficiency disorders, except human immunodeficiency virus [HIV] disease
- sarcoidosis
Inclusion terms
Conditions classified to this code.
- Multiple carboxylase deficiency