2023

  1. ICD-10-CM Codes
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  2. D50-D89
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  3. D80-D89
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  4. D84-
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  5. 2023 ICD-10-CM Diagnosis Code D84.1

2023 ICD-10-CM Diagnosis Code D84.1

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Defects in the complement system

    2016 2017 2018 2019 2020 2021 2022 2023 Billable/Specific Code
  • D84.1 is a billable/specific ICD-10-CM code that can be used to indicate a diagnosis for reimbursement purposes.
  • The 2023 edition of ICD-10-CM D84.1 became effective on October 1, 2022.
  • This is the American ICD-10-CM version of D84.1 - other international versions of ICD-10 D84.1 may differ.
Applicable To
  • C1 esterase inhibitor [C1-INH] deficiency
The following code(s) above D84.1 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 D84.1:
  • D50-D89
    2023 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
    2023 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
Approximate Synonyms
  • Angioedema, hereditary
  • Circulating enzyme deficiency
  • Complement 4 deficiency
  • Complement deficiency c4
  • Complement deficiency disease
  • Deficiency of circulating enzyme
  • Hereditary angioneurotic edema
  • Hypocomplementemia
Clinical Information
  • Rare disease where complement protein(s) are absent or in diminished amount relative to the normal requirement of an organism; associated with autoimmune disease or increased susceptibility to infections, problems encountered depend on which pathway is affected.
ICD-10-CM D84.1 is grouped within Diagnostic Related Group(s) (MS-DRG v40.0):
  • 642 Inborn and other disorders of metabolism

Convert D84.1 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
  • 2022 (effective 10/1/2021): No change
  • 2023 (effective 10/1/2022): No change
Code annotations containing back-references to D84.1:
  • Code Also: D59.32
    ICD-10-CM Diagnosis Code D59.32

    Hereditary hemolytic-uremic syndrome

      2023 - New Code Billable/Specific Code
    Applicable To
    • Atypical hemolytic uremic syndrome with an identified genetic cause
    Code Also
    • , if applicable:
    • defects in the complement system (D84.1)
    • methylmalonic acidemia (E71.120)
  • Type 1 Excludes: D84.81
    , L50
    ICD-10-CM Diagnosis Code D84.81

    Immunodeficiency due to conditions classified elsewhere

      2021 - New Code 2022 2023 Billable/Specific Code Manifestation Code
    Code First
    • underlying condition, such as:
    • chromosomal abnormalities (Q90-Q99)
    • diabetes mellitus (E08-E13)
    • malignant neoplasms (C00-C96)
    Type 1 Excludes
    • certain disorders involving the immune mechanism (D80-D83, D84.0, D84.1, D84.9)
    • human immunodeficiency virus [HIV] disease (B20)
    ICD-10-CM Diagnosis Code L50

    Urticaria

      2016 2017 2018 2019 2020 2021 2022 2023 Non-Billable/Non-Specific Code
    Type 1 Excludes
    • allergic contact dermatitis (L23.-)
    • angioneurotic edema (T78.3)
    • giant urticaria (T78.3)
    • hereditary angio-edema (D84.1)
    • Quincke's edema (T78.3)
    • serum urticaria (T80.6-)
    • solar urticaria (L56.3)
    • urticaria neonatorum (P83.8)
    • urticaria papulosa (L28.2)
    • urticaria pigmentosa (D47.01)

Diagnosis Index entries containing back-references to D84.1:
  • Angioedema (allergic) (any site) (with urticaria) T78.3
    ICD-10-CM Diagnosis Code T78.3

    Angioneurotic edema

      2016 2017 2018 2019 2020 2021 2022 2023 Non-Billable/Non-Specific Code
    Applicable To
    • Allergic angioedema
    • Giant urticaria
    • Quincke's edema
    Type 1 Excludes
    • hereditary D84.1
  • Angioneurotic edema (allergic) (any site) (with urticaria) T78.3
    ICD-10-CM Diagnosis Code T78.3

    Angioneurotic edema

      2016 2017 2018 2019 2020 2021 2022 2023 Non-Billable/Non-Specific Code
    Applicable To
    • Allergic angioedema
    • Giant urticaria
    • Quincke's edema
    Type 1 Excludes
    • hereditary D84.1
  • Bannister's disease T78.3
    ICD-10-CM Diagnosis Code T78.3

    Angioneurotic edema

      2016 2017 2018 2019 2020 2021 2022 2023 Non-Billable/Non-Specific Code
    Applicable To
    • Allergic angioedema
    • Giant urticaria
    • Quincke's edema
    Type 1 Excludes
    • hereditary D84.1
  • Defect, defective Q89.9
    ICD-10-CM Diagnosis Code Q89.9

    Congenital malformation, unspecified

      2016 2017 2018 2019 2020 2021 2022 2023 Billable/Specific Code POA Exempt
    Applicable To
    • Congenital anomaly NOS
    • Congenital deformity NOS
    • complement system D84.1
  • Deficiency, deficient
    • C1 esterase inhibitor D84.1 (C1-INH)
  • Edema, edematous (infectious) (pitting) (toxic) R60.9
    ICD-10-CM Diagnosis Code R60.9

    Edema, unspecified

      2016 2017 2018 2019 2020 2021 2022 2023 Billable/Specific Code
    Applicable To
    • Fluid retention NOS
    • angioneurotic (allergic) (any site) (with urticaria) T78.3
      ICD-10-CM Diagnosis Code T78.3

      Angioneurotic edema

        2016 2017 2018 2019 2020 2021 2022 2023 Non-Billable/Non-Specific Code
      Applicable To
      • Allergic angioedema
      • Giant urticaria
      • Quincke's edema
      Type 1 Excludes
      • hereditary D84.1
    • circumscribed, acute T78.3
      ICD-10-CM Diagnosis Code T78.3

      Angioneurotic edema

        2016 2017 2018 2019 2020 2021 2022 2023 Non-Billable/Non-Specific Code
      Applicable To
      • Allergic angioedema
      • Giant urticaria
      • Quincke's edema
      Type 1 Excludes
      • hereditary D84.1
    • essential, acute T78.3
      ICD-10-CM Diagnosis Code T78.3

      Angioneurotic edema

        2016 2017 2018 2019 2020 2021 2022 2023 Non-Billable/Non-Specific Code
      Applicable To
      • Allergic angioedema
      • Giant urticaria
      • Quincke's edema
      Type 1 Excludes
      • hereditary D84.1
    • glottis, glottic, glottidis (obstructive) (passive) J38.4
      ICD-10-CM Diagnosis Code J38.4

      Edema of larynx

        2016 2017 2018 2019 2020 2021 2022 2023 Billable/Specific Code
      Applicable To
      • Edema (of) glottis
      • Subglottic edema
      • Supraglottic edema
      Type 1 Excludes
      • acute obstructive laryngitis [croup] (J05.0)
      • edematous laryngitis (J04.0)
      • allergic T78.3
        ICD-10-CM Diagnosis Code T78.3

        Angioneurotic edema

          2016 2017 2018 2019 2020 2021 2022 2023 Non-Billable/Non-Specific Code
        Applicable To
        • Allergic angioedema
        • Giant urticaria
        • Quincke's edema
        Type 1 Excludes
        • hereditary D84.1
    • periodic T78.3
      ICD-10-CM Diagnosis Code T78.3

      Angioneurotic edema

        2016 2017 2018 2019 2020 2021 2022 2023 Non-Billable/Non-Specific Code
      Applicable To
      • Allergic angioedema
      • Giant urticaria
      • Quincke's edema
      Type 1 Excludes
      • hereditary D84.1
    • Quincke's T78.3
      ICD-10-CM Diagnosis Code T78.3

      Angioneurotic edema

        2016 2017 2018 2019 2020 2021 2022 2023 Non-Billable/Non-Specific Code
      Applicable To
      • Allergic angioedema
      • Giant urticaria
      • Quincke's edema
      Type 1 Excludes
      • hereditary D84.1
  • Giant
    • urticaria T78.3
      ICD-10-CM Diagnosis Code T78.3

      Angioneurotic edema

        2016 2017 2018 2019 2020 2021 2022 2023 Non-Billable/Non-Specific Code
      Applicable To
      • Allergic angioedema
      • Giant urticaria
      • Quincke's edema
      Type 1 Excludes
      • hereditary D84.1
  • Quincke's disease or edema T78.3
    ICD-10-CM Diagnosis Code T78.3

    Angioneurotic edema

      2016 2017 2018 2019 2020 2021 2022 2023 Non-Billable/Non-Specific Code
    Applicable To
    • Allergic angioedema
    • Giant urticaria
    • Quincke's edema
    Type 1 Excludes
    • hereditary D84.1
  • Urticaria L50.9
    ICD-10-CM Diagnosis Code L50.9

    Urticaria, unspecified

      2016 2017 2018 2019 2020 2021 2022 2023 Billable/Specific Code
    • with angioneurotic edema T78.3
      ICD-10-CM Diagnosis Code T78.3

      Angioneurotic edema

        2016 2017 2018 2019 2020 2021 2022 2023 Non-Billable/Non-Specific Code
      Applicable To
      • Allergic angioedema
      • Giant urticaria
      • Quincke's edema
      Type 1 Excludes
      • hereditary D84.1
    • giant T78.3
      ICD-10-CM Diagnosis Code T78.3

      Angioneurotic edema

        2016 2017 2018 2019 2020 2021 2022 2023 Non-Billable/Non-Specific Code
      Applicable To
      • Allergic angioedema
      • Giant urticaria
      • Quincke's edema
      Type 1 Excludes
      • hereditary D84.1
    • larynx T78.3
      ICD-10-CM Diagnosis Code T78.3

      Angioneurotic edema

        2016 2017 2018 2019 2020 2021 2022 2023 Non-Billable/Non-Specific Code
      Applicable To
      • Allergic angioedema
      • Giant urticaria
      • Quincke's edema
      Type 1 Excludes
      • hereditary D84.1

ICD-10-CM Codes Adjacent To D84.1
D82.8 Immunodeficiency associated with other specified major defects
D82.9 Immunodeficiency associated with major defect, unspecified
D83 Common variable immunodeficiency
D83.0 Common variable immunodeficiency with predominant abnormalities of B-cell numbers and function
D83.1 Common variable immunodeficiency with predominant immunoregulatory T-cell disorders
D83.2 Common variable immunodeficiency with autoantibodies to B- or T-cells
D83.8 Other common variable immunodeficiencies
D83.9 Common variable immunodeficiency, unspecified
D84 Other immunodeficiencies
D84.0 Lymphocyte function antigen-1 [LFA-1] defect
D84.1 Defects in the complement system
D84.8 Other specified immunodeficiencies
D84.81 Immunodeficiency due to conditions classified elsewhere
D84.82 Immunodeficiency due to drugs and external causes
D84.821 Immunodeficiency due to drugs
D84.822 Immunodeficiency due to external causes
D84.89 Other immunodeficiencies
D84.9 Immunodeficiency, unspecified
D86 Sarcoidosis
D86.0 Sarcoidosis of lung
D86.1 Sarcoidosis of lymph nodes

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

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