Other and unspecified metabolic disorders E88-

Use Additional
Use Additional Help
Certain conditions have both an underlying etiology and multiple body system manifestations due to the underlying etiology. For such conditions the ICD-10-CM has a coding convention that requires the underlying condition be sequenced first followed by the manifestation. Wherever such a combination exists there is a "use additional code" note at the etiology code, and a "code first" note at the manifestation code. These instructional notes indicate the proper sequencing order of the codes, etiology followed by manifestation. In most cases the manifestation codes will have in the code title, "in diseases classified elsewhere." Codes with this title are a component of the etiology/manifestation convention. The code title indicates that it is a manifestation code. "In diseases classified elsewhere" codes are never permitted to be used as first listed or principle diagnosis codes. They must be used in conjunction with an underlying condition code and they must be listed following the underlying condition.
  • codes for associated conditions
Type 1 Excludes
Type 1 Excludes Help
A type 1 excludes note is a pure excludes. It means "not coded here". A type 1 excludes note indicates that the code excluded should never be used at the same time as E88. A type 1 excludes note is for used for when two conditions cannot occur together, such as a congenital form versus an acquired form of the same condition.
  • histiocytosis X (chronic) (
    ICD-10-CM Diagnosis Code C96.6

    Unifocal Langerhans-cell histiocytosis

      2016 2017 2018 2019 2020 2021 2022 2023 Billable/Specific Code
    Applicable To
    • Eosinophilic granuloma
    • Histiocytosis X, unifocal
    • Histiocytosis X NOS
    • Langerhans-cell histiocytosis NOS
    Type 1 Excludes
    • multifocal and multisysemic (disseminated) Langerhans-cell histiocytosis (C96.0)
    • multifocal and unisystemic Langerhans-cell histiocytosis (C96.5)
  • E88 Other and unspecified metabolic disorders
    • E88.0 Disorders of plasma-protein metabolism, not elsewhere classified
      • E88.01 Alpha-1-antitrypsin deficiency
      • E88.02 Plasminogen deficiency
      • E88.09 Other disorders of plasma-protein metabolism, not elsewhere classified
    • E88.1 Lipodystrophy, not elsewhere classified
    • E88.2 Lipomatosis, not elsewhere classified
    • E88.3 Tumor lysis syndrome
    • E88.4 Mitochondrial metabolism disorders
      • E88.40 Mitochondrial metabolism disorder, unspecified
      • E88.41 MELAS syndrome
      • E88.42 MERRF syndrome
      • E88.49 Other mitochondrial metabolism disorders
    • E88.8 Other specified metabolic disorders
      • E88.81 Metabolic syndrome
      • E88.89 Other specified metabolic disorders
    • E88.9 Metabolic disorder, unspecified