2021 ICD-10-CM Diagnosis Code D81.810

Biotinidase deficiency

    2016 2017 2018 2019 2020 2021 Billable/Specific Code
  • D81.810 is a billable/specific ICD-10-CM code that can be used to indicate a diagnosis for reimbursement purposes.
  • The 2021 edition of ICD-10-CM D81.810 became effective on October 1, 2020.
  • This is the American ICD-10-CM version of D81.810 - other international versions of ICD-10 D81.810 may differ.
The following code(s) above D81.810 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 D81.810:
  • D50-D89
    2021 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
    2021 ICD-10-CM Range D80-D89

    Certain disorders involving the immune mechanism

    • 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 2021 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 2021 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
Clinical Information
  • A genetic disorder caused by mutations in the btd gene. It is characterized by reduced or absent activity of the enzyme biotinidase which is responsible for the recycling of the vitamin biotin. Signs and symptoms appear in childhood and include seizures, hypotonia and developmental delays. If left untreated, it leads to vision and hearing loss, infections, alopecia and ataxia.
  • The late onset form of multiple carboxylase deficiency (deficiency of the activities of biotin-dependent enzymes propionyl-coa carboxylase, methylcrotonyl-coa carboxylase, and pyruvate carboxylase) due to a defect or deficiency in biotinidase which is essential for recycling biotin.
ICD-10-CM D81.810 is grouped within Diagnostic Related Group(s) (MS-DRG v38.0):
  • 642 Inborn and other disorders of metabolism

Convert D81.810 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
  • 2021 (effective 10/1/2020): No change

Diagnosis Index entries containing back-references to D81.810:
  • Deficiency, deficient
    • biotinidase D81.810
  • Immunodeficiency D84.9
    ICD-10-CM Diagnosis Code D84.9

    Immunodeficiency, unspecified

      2016 2017 2018 2019 2020 2021 Billable/Specific Code
    Applicable To
    • Immunocompromised NOS
    • Immunodeficient NOS
    • Immunosuppressed NOS
    • combined D81.9
      ICD-10-CM Diagnosis Code D81.9

      Combined immunodeficiency, unspecified

        2016 2017 2018 2019 2020 2021 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 2021 Billable/Specific Code
        Applicable To
        • Multiple carboxylase deficiency, unspecified
        • biotinidase D81.810

ICD-10-CM Codes Adjacent To D81.810
D81.30 Adenosine deaminase deficiency, unspecified
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

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