D81.818

Other biotin-dependent carboxylase deficiency

Billable code

This code does not map to a risk-adjusting HCC in the loaded models.

Coding notes

Excludes 1 never coded together · Excludes 2 may co-exist

This code · D81.818
Synonyms

Holocarboxylase synthetase deficiency Other multiple carboxylase deficiency

Inherited from D81.81Biotin-dependent carboxylase deficiency
Excludes 1

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

Synonyms

Multiple carboxylase deficiency

Inherited from D81Combined immunodeficiencies
Excludes 1

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

Inherited from D80-D89Certain disorders involving the immune mechanism
Includes

defects in the complement system immunodeficiency disorders, except human immunodeficiency virus [HIV] disease sarcoidosis

Excludes 1

autoimmune disease (systemic) NOS (M35.9) functional disorders of polymorphonuclear neutrophils (D71-) human immunodeficiency virus [HIV] disease (B20)

Inherited from chapter 3Diseases of the blood and blood-forming organs and certain disorders involving the immune mechanism
Excludes 2

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)

Official Guidelines for D81.818

FY 2026 ›
I.C.3Chapter-specific guidanceChapter 3: Disease of the blood and blood-forming organs and certain disorders involving the immune mechanism (D50-D89)

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