2020 ICD-10-CM Diagnosis Code E70.29

Other disorders of tyrosine metabolism

    2016 2017 2018 2019 2020 Billable/Specific Code
  • E70.29 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 E70.29 became effective on October 1, 2019.
  • This is the American ICD-10-CM version of E70.29 - other international versions of ICD-10 E70.29 may differ.
Applicable To
  • Alkaptonuria
  • Ochronosis
The following code(s) above E70.29 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 E70.29:
  • E00-E89
    2020 ICD-10-CM Range E00-E89

    Endocrine, nutritional and metabolic diseases

    Note
    • All neoplasms, whether functionally active or not, are classified in Chapter 2. Appropriate codes in this chapter (i.e. E05.8, E07.0, E16-E31, E34.-) may be used as additional codes to indicate either functional activity by neoplasms and ectopic endocrine tissue or hyperfunction and hypofunction of endocrine glands associated with neoplasms and other conditions classified elsewhere.
    Type 1 Excludes
    • transitory endocrine and metabolic disorders specific to newborn (P70-P74)
    Endocrine, nutritional and metabolic diseases
  • E70-E88
    2020 ICD-10-CM Range E70-E88

    Metabolic disorders

    Type 1 Excludes
    • androgen insensitivity syndrome (E34.5-)
    • congenital adrenal hyperplasia (E25.0)
    • Ehlers-Danlos syndrome (Q79.6-)
    • hemolytic anemias attributable to enzyme disorders (D55.-)
    • Marfan's syndrome (Q87.4)
    • 5-alpha-reductase deficiency (E29.1)
    Metabolic disorders
  • E70.2
    ICD-10-CM Diagnosis Code E70.2

    Disorders of tyrosine metabolism

      2016 2017 2018 2019 2020 Non-Billable/Non-Specific Code
    Type 1 Excludes
    • transitory tyrosinemia of newborn (P74.5)
    Disorders of tyrosine metabolism
Approximate Synonyms
  • Alcaptonuric ochronosis
  • Alkaptonuria
  • Ochronosis
Clinical Information
  • A disorder characterized by bluish-black discoloration of the cartilaginous tissues due to accumulation of homogentisic acid. It is associated with alkaptonuria. Signs and symptoms include dark urine, skin pigmentation, and arthritis.
  • A rare autosomal recessive disorder characterized by abnormalities in the metabolism of phenylalanine and tyrosine. It results in the accumulation in the blood of homogentisic acid which is excreted in the urine. The presence of homogentisic acid in the urine causes its color to turn black. The excessive amount of homogentisic acid in the blood may cause damage to cartilage and heart valves, and may result in the formation of kidney stones.
  • An inborn error of amino acid metabolism resulting from a defect in the enzyme homogentisate 1,2-dioxygenase, an enzyme involved in the breakdown of phenylalanine and tyrosine. It is characterized by accumulation of homogentisic acid in the urine, ochronosis in various tissues, and arthritis.
  • The yellowish discoloration of connective tissue due to deposition of homogentisic acid (a brown-black pigment). This is due to defects in the metabolism of phenylalanine and tyrosine. Ochronosis occurs in alkaptonuria, but has also been associated with exposure to certain chemicals (e.g., phenol, trinitrophenol, benzene derivatives).
  • What: ochronosis. Ochronosis: the pathologic accumulation of a blue-black pigment in the connective tissues of persons with alkaptonuria, a rare inborn error of metabolism. These persons lack the enzyme homogentisic acid oxidase, resulting in a buildup of homogentisic acid, a precursor of the pigment. Why: the great majority of patients with ochronosis eventually develop an arthropathy clinically resembling degenerative joint disease. How: ochronosis is diagnosed by the combination of pigmentation of the cartilage, the presence of homogentisic acid in the urine, and arthritis usually involving the knees, hips, lumbosacral spine, and shoulders. The slate blue-black pigmentation is most apparent in the sclera of the eyes, the external ears, and the tympanic membranes. The cerumen may also show a dark discoloration. The presence of homogentisic acid in the urine is determined by the dark color which occurs when the urine is alkalinized and by paper chromatography. Refs: schumacher hr and holdsworth de: ochronotic arthropathy i. Clinico-pathologic studies. Semin arthritis rheum 6:207, 1977. Dn19296-1.
ICD-10-CM E70.29 is grouped within Diagnostic Related Group(s) (MS-DRG v37.0):
  • 642 Inborn and other disorders of metabolism

Convert E70.29 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 E70.29:
  • Alcaptonuria E70.29
  • Alkaptonuria E70.29
  • Arthritis, arthritic (acute) (chronic) (nonpyogenic) (subacute) M19.90
    ICD-10-CM Diagnosis Code M19.90

    Unspecified osteoarthritis, unspecified site

      2016 2017 2018 2019 2020 Billable/Specific Code
    Applicable To
    • Arthrosis NOS
    • Arthritis NOS
    • Osteoarthritis NOS
    • in (due to)
      • ochronosis E70.29 - see also subcategory M14.8-
    • ochronotic E70.29 - see also subcategory M14.8-
  • Deficiency, deficient
    • homogentisate 1,2-dioxygenase E70.29
  • Disease, diseased - see also Syndrome
    • connective tissue, systemic (diffuse) M35.9
      ICD-10-CM Diagnosis Code M35.9

      Systemic involvement of connective tissue, unspecified

        2016 2017 2018 2019 2020 Billable/Specific Code
      Applicable To
      • Autoimmune disease (systemic) NOS
      • Collagen (vascular) disease NOS
      • in (due to)
        • ochronosis E70.29
  • Disorder (of) - see also Disease
    • metabolism NOS E88.9
      ICD-10-CM Diagnosis Code E88.9

      Metabolic disorder, unspecified

        2016 2017 2018 2019 2020 Billable/Specific Code
      • amino-acid E72.9
        ICD-10-CM Diagnosis Code E72.9

        Disorder of amino-acid metabolism, unspecified

          2016 2017 2018 2019 2020 Billable/Specific Code
        • aromatic E70.9
          ICD-10-CM Diagnosis Code E70.9

          Disorder of aromatic amino-acid metabolism, unspecified

            2016 2017 2018 2019 2020 Billable/Specific Code
          • tyrosine E70.20
            ICD-10-CM Diagnosis Code E70.20

            Disorder of tyrosine metabolism, unspecified

              2016 2017 2018 2019 2020 Billable/Specific Code
            • other specified E70.29
  • Homogentisate 1,2-dioxygenase deficiency E70.29
  • Ochronosis E70.29 (endogenous)
  • Osteoarthrosis (degenerative) (hypertrophic) (joint) - see also Osteoarthritis
    • deformans alkaptonurica E70.29
  • Tyrosinuria E70.29

ICD-10-CM Codes Adjacent To E70.29
E67.2 Megavitamin-B6 syndrome
E67.3 Hypervitaminosis D
E67.8 Other specified hyperalimentation
E68 Sequelae of hyperalimentation
E70 Disorders of aromatic amino-acid metabolism
E70.0 Classical phenylketonuria
E70.1 Other hyperphenylalaninemias
E70.2 Disorders of tyrosine metabolism
E70.20 Disorder of tyrosine metabolism, unspecified
E70.21 Tyrosinemia
E70.29 Other disorders of tyrosine metabolism
E70.3 Albinism
E70.30 …… unspecified
E70.31 Ocular albinism
E70.310 X-linked ocular albinism
E70.311 Autosomal recessive ocular albinism
E70.318 Other ocular albinism
E70.319 …… unspecified
E70.32 Oculocutaneous albinism
E70.320 Tyrosinase negative oculocutaneous albinism
E70.321 Tyrosinase positive oculocutaneous albinism

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