D81.818

Other biotin-dependent carboxylase deficiency

Billable / specific

D81.818 is a billable, specific ICD-10-CM code that can be used to indicate a diagnosis for reimbursement purposes in FY2027.

Inclusion terms

Applicable to

  • Holocarboxylase synthetase deficiency
  • Other multiple carboxylase deficiency

Includes (from D81)

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

Excludes1

Not coded here — never report together with this code.

  • biotin-dependent carboxylase deficiency due to dietary deficiency of biotin (E53.8) (from D81.81)
  • autoimmune disease (systemic) NOS (M35.9) (from D81)
  • functional disorders of polymorphonuclear neutrophils (D71-) (from D81)
  • autosomal recessive agammaglobulinemia (Swiss type) (D80.0) (from D81)

Excludes2

Not included here — may be reported together when both conditions exist.

  • autoimmune disease (systemic) NOS (M35.9) (from D81)
  • certain conditions originating in the perinatal period (P00-P96) (from D81)
  • complications of pregnancy, childbirth and the puerperium (O00-O9A) (from D81)
  • congenital malformations, deformations and chromosomal abnormalities (Q00-Q99) (from D81)
  • endocrine, nutritional and metabolic diseases (E00-E88) (from D81)
  • human immunodeficiency virus [HIV] disease (B20) (from D81)
  • injury, poisoning and certain other consequences of external causes (S00-T88) (from D81)
  • neoplasms (C00-D49) (from D81)
  • symptoms, signs and abnormal clinical and laboratory findings, not elsewhere classified (R00-R94) (from D81)
  • human immunodeficiency virus [HIV] disease (B20) (from D81)

Also known as

  • Holocarboxylase synthetase deficiency
  • Other multiple carboxylase deficiency
  • Immunodeficiency, combined, biotin-dependent carboxylase, holocarboxylase synthetase
  • Immunodeficiency, combined, biotin-dependent carboxylase, specified type NEC

Parent code

  • D81.81Biotin-dependent carboxylase deficiencyHeader

Related codes

  • D81.810Biotinidase deficiencyBillable
  • D81.819Biotin-dependent carboxylase deficiency, unspecifiedBillable

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