2020 ICD-10-CM Diagnosis Code D81.818

Other biotin-dependent carboxylase deficiency

    2016 2017 2018 2019 2020 Billable/Specific Code
  • D81.818 is a billable/specific ICD-10-CM code that can be used to indicate a diagnosis for reimbursement purposes.
  • The 2020 edition of ICD-10-CM D81.818 became effective on October 1, 2019.
  • This is the American ICD-10-CM version of D81.818 - other international versions of ICD-10 D81.818 may differ.
Applicable To
  • Holocarboxylase synthetase deficiency
  • Other multiple carboxylase deficiency
The following code(s) above D81.818 contain annotation back-references
Annotation Back-References
In this context, annotation back-references refer to codes that contain:
  • Applicable To annotations, or
  • Code Also annotations, or
  • Code First annotations, or
  • Excludes1 annotations, or
  • Excludes2 annotations, or
  • Includes annotations, or
  • Note annotations, or
  • Use Additional annotations
that may be applicable to D81.818:
  • D50-D89
    2020 ICD-10-CM Range D50-D89

    Diseases of the blood and blood-forming organs and certain disorders involving the immune mechanism

    Type 2 Excludes
    • 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)
    Diseases of the blood and blood-forming organs and certain disorders involving the immune mechanism
  • D80-D89
    2020 ICD-10-CM Range D80-D89

    Certain disorders involving the immune mechanism

    Includes
    • defects in the complement system
    • immunodeficiency disorders, except human immunodeficiency virus [HIV] disease
    • sarcoidosis
    Type 1 Excludes
    • autoimmune disease (systemic) NOS (M35.9)
    • functional disorders of polymorphonuclear neutrophils (D71)
    • human immunodeficiency virus [HIV] disease (B20)
    Certain disorders involving the immune mechanism
  • D81
    ICD-10-CM Diagnosis Code D81

    Combined immunodeficiencies

      2016 2017 2018 2019 2020 Non-Billable/Non-Specific Code
    Type 1 Excludes
    • autosomal recessive agammaglobulinemia (Swiss type) (D80.0)
    Combined immunodeficiencies
  • D81.81
    ICD-10-CM Diagnosis Code D81.81

    Biotin-dependent carboxylase deficiency

      2016 2017 2018 2019 2020 Non-Billable/Non-Specific Code
    Applicable To
    • Multiple carboxylase deficiency
    Type 1 Excludes
    • biotin-dependent carboxylase deficiency due to dietary deficiency of biotin (E53.8)
    Biotin-dependent carboxylase deficiency
Approximate Synonyms
  • Deficiency, holocarboxylase synthease
  • Holocarboxylase synthase deficiency
Clinical Information
  • A rare autosomal recessive inherited disorder caused by mutations in the hlcs gene. It is characterized by deficiency of the enzyme holocarboxylase synthetase which facilitates the effective use of the vitamin biotin in the body. Signs and symptoms appear early in life and include breathing difficulties, feeding difficulties, alopecia, skin rash, and lethargy. Lifelong administration of biotin supplements is required. If it is not treated properly, it may lead to developmental delays, seizures, and coma.
  • A rare autosomal recessive inherited disorder that is manifested in the neonatal period and is caused by mutations in the hlcs gene. It is characterized by deficiency of the enzyme holocarboxylase synthetase which facilitates the effective use of the vitamin biotin in the body. Signs and symptoms include difficulties in feeding and breathing, skin rash, seizures, lethargy, and coma.
  • The neonatal form of multiple carboxylase deficiency that is caused by a defect or deficiency in holocarboxylase synthetase. Hlcs is the enzyme that covalently links biotin to the biotin dependent carboxylases (propionyl-coa-carboxylase, pyruvate carboxylase, and beta-methylcrotonyl-coa carboxylase).
ICD-10-CM D81.818 is grouped within Diagnostic Related Group(s) (MS-DRG v37.0):
  • 640 Miscellaneous disorders of nutrition, metabolism, fluids and electrolytes with mcc
  • 641 Miscellaneous disorders of nutrition, metabolism, fluids and electrolytes without mcc

Convert D81.818 to ICD-9-CM

Code History
  • 2016 (effective 10/1/2015): New code (first year of non-draft ICD-10-CM)
  • 2017 (effective 10/1/2016): No change
  • 2018 (effective 10/1/2017): No change
  • 2019 (effective 10/1/2018): No change
  • 2020 (effective 10/1/2019): No change

Diagnosis Index entries containing back-references to D81.818:
  • Immunodeficiency D84.9
    ICD-10-CM Diagnosis Code D84.9

    Immunodeficiency, unspecified

      2016 2017 2018 2019 2020 Billable/Specific Code
    • combined D81.9
      ICD-10-CM Diagnosis Code D81.9

      Combined immunodeficiency, unspecified

        2016 2017 2018 2019 2020 Billable/Specific Code
      Applicable To
      • Severe combined immunodeficiency disorder [SCID] NOS
      • biotin-dependent carboxylase D81.819
        ICD-10-CM Diagnosis Code D81.819

        Biotin-dependent carboxylase deficiency, unspecified

          2016 2017 2018 2019 2020 Billable/Specific Code
        Applicable To
        • Multiple carboxylase deficiency, unspecified
        • holocarboxylase synthetase D81.818
        • specified type NEC D81.818

ICD-10-CM Codes Adjacent To D81.818
D81.31 Severe combined immunodeficiency due to adenosine deaminase deficiency
D81.32 Adenosine deaminase 2 deficiency
D81.39 Other adenosine deaminase deficiency
D81.4 Nezelof's syndrome
D81.5 Purine nucleoside phosphorylase [PNP] deficiency
D81.6 Major histocompatibility complex class I deficiency
D81.7 Major histocompatibility complex class II deficiency
D81.8 Other combined immunodeficiencies
D81.81 Biotin-dependent carboxylase deficiency
D81.810 Biotinidase deficiency
D81.818 Other biotin-dependent carboxylase deficiency
D81.819 …… unspecified
D81.89 Other combined immunodeficiencies
D81.9 Combined immunodeficiency, unspecified
D82 Immunodeficiency associated with other major defects
D82.0 Wiskott-Aldrich syndrome
D82.1 Di George's syndrome
D82.2 Immunodeficiency with short-limbed stature
D82.3 Immunodeficiency following hereditary defective response to Epstein-Barr virus
D82.4 Hyperimmunoglobulin E [IgE] syndrome
D82.8 Immunodeficiency associated with other specified major defects

Reimbursement claims with a date of service on or after October 1, 2015 require the use of ICD-10-CM codes.