DxCodebookFY2027 · Oct 1, 2026 – Sep 30, 2027

Category · FY2027

E88 Other and unspecified metabolic disorders

  • Header · not billable

E88 is not billable. The category heads 24 billable codes in FY2027, grouped under 8 subdivisions.

Index → code

Where it sits

  1. Chapter 4 Endocrine, nutritional and metabolic diseases (E00-E89)
  2. E70-E88 Metabolic disorders (E70-E88)
  3. E88 Other and unspecified metabolic disorders
Billable codes
24
Subdivisions
8
CC / MCC codes
6 / 1
HCC V28 codes
2
New FY2027
0
Index entries
122

Codes in E88

  1. E88.0
    Disorders of plasma-protein metabolism, not elsewhere classified
    Header
    Excludes1 3, Excludes2 1

    Excludes1

    • monoclonal gammopathy (of undetermined significance) (D47.2) D47.2
    • polyclonal hypergammaglobulinemia (D89.0) D89.0
    • Waldenström macroglobulinemia (C88.00) C88.00

    Excludes2

    • disorder of lipoprotein metabolism (E78.-) E78
  2. E88.01
    Alpha-1-antitrypsin deficiency
    Billable50
    Inclusion terms 1

    Inclusion terms

    • AAT deficiency
  3. E88.02
    Plasminogen deficiency
    BillableCC
    Inclusion terms 4, Use additional code 4, Code also 1

    Inclusion terms

    • Dysplasminogenemia
    • Hypoplasminogenemia
    • Type 1 plasminogen deficiency
    • Type 2 plasminogen deficiency

    Use additional code

    • code for associated findings, such as:
    • hydrocephalus (G91.4) G91.4
    • otitis media (H67.-) H67
    • respiratory disorder related to plasminogen deficiency (J99) J99

    Code also

    • , if applicable, ligneous conjunctivitis (H10.51-) H10.51
  4. E88.09
    Other disorders of plasma-protein metabolism, not elsewhere classified
    Billable
    Inclusion terms 1

    Inclusion terms

    • Bisalbuminemia
  5. E88.1
    Lipodystrophy, not elsewhere classified
    Header
    Excludes1 1

    Excludes1

    • Whipple's disease (K90.81) K90.81
  6. E88.10
    Lipodystrophy, unspecified
    Billable
    Inclusion terms 1

    Inclusion terms

    • Lipodystrophy NOS
  7. E88.11
    Partial lipodystrophy
    Billable
    Inclusion terms 3

    Inclusion terms

    • Acquired partial lipodystrophy (APL)
    • Barraquer-Simons lipodystrophy
    • Familial partial lipodystrophy (FPLD)
  8. E88.12
    Generalized lipodystrophy
    Billable
    Inclusion terms 4

    Inclusion terms

    • Acquired generalized lipodystrophy (AGL)
    • Berardinelli-Siep syndrome
    • Congenital generalized lipodystrophy (CGL)
    • Lawrence syndrome
  9. E88.13
    Localized lipodystrophy
    Billable
    Inclusion terms 2

    Inclusion terms

    • Injection lipodystrophy
    • Insulin lipodystrophy
  10. E88.14
    HIV-associated lipodystrophy
    Billable
    Code first 1, Use additional code 1

    Code first

    • any human immunodeficiency virus [HIV] disease (B20) B20

    Use additional code

    • code for adverse effect, if applicable, to identify drug (T37.5X5-) T37.5X5
  11. E88.19
    Other lipodystrophy, not elsewhere classified
    Billable
  12. E88.2
    Lipomatosis, not elsewhere classified
    Billable
    Inclusion terms 2

    Inclusion terms

    • Lipomatosis NOS
    • Lipomatosis (Check) dolorosa [Dercum]
  13. E88.3
    Tumor lysis syndrome
    BillableMCC
    Inclusion terms 2, Use additional code 1

    Inclusion terms

    • Tumor lysis syndrome (spontaneous)
    • Tumor lysis syndrome following antineoplastic drug chemotherapy

    Use additional code

    • code for adverse effect, if applicable, to identify drug (T45.1X5) T45.1X5
  14. E88.4
    Mitochondrial metabolism disorders
    Header
    Excludes1 6

    Excludes1

    • disorders of pyruvate metabolism (E74.4) E74.4
    • Kearns-Sayre syndrome (H49.81) H49.81
    • Leber's disease (H47.22) H47.22
    • Leigh's encephalopathy (G31.82) G31.82
    • Mitochondrial myopathy, NEC (G71.3) G71.3
    • Reye's syndrome (G93.7) G93.7
  15. E88.40
    Mitochondrial metabolism disorder, unspecified
    BillableCC
  16. E88.41
    MELAS syndrome
    BillableCC
    Inclusion terms 1

    Inclusion terms

    • Mitochondrial myopathy, encephalopathy, lactic acidosis and stroke-like episodes
  17. E88.42
    MERRF syndrome
    BillableCC
    Inclusion terms 1, Code also 1

    Inclusion terms

    • Myoclonic epilepsy associated with ragged-red fibers

    Code also

    • progressive myoclonic epilepsy (G40.3-) G40.3
  18. E88.43
    Disorders of mitochondrial tRNA synthetases
    BillableCC
    Inclusion terms 6, Code also 2

    Inclusion terms

    • ARS2-related mitochondrial disorders
    • LBSL
    • Leukoencephalopathy with brainstem - spinal cord involvement - lactate elevation
    • Leukoencephalopathy with thalamus - brainstem involvement - high lactate
    • LTBL
    • Mitochondrial aminoacyl-tRNA synthetase disorders

    Code also

    • , if applicable, associated condition such as:
    • leukoencephalopathy (G93.49) G93.49
  19. E88.49
    Other mitochondrial metabolism disorders
    BillableCC
  20. E88.8
    Other specified metabolic disorders
    Header
  21. E88.81
    Metabolic syndrome and other insulin resistance
    Header
    Use additional code 2

    Use additional code

    • codes for associated manifestations, such as:
    • obesity (E66.-) E66
  22. E88.810
    Metabolic syndrome
    Billable
    Inclusion terms 1

    Inclusion terms

    • Dysmetabolic syndrome
  23. E88.811
    Insulin resistance syndrome, Type A
    Billable
  24. E88.818
    Other insulin resistance
    Billable
    Inclusion terms 1

    Inclusion terms

    • Insulin resistance syndrome, Type B
  25. E88.819
    Insulin resistance, unspecified
    Billable
  26. E88.82
    Obesity due to disruption of MC4R pathway
    Billable
    Use additional code 2

    Use additional code

    • code, if applicable, to identify associated manifestations, such as polyphagia (R63.2) R63.2
    • code to identify body mass index (BMI), if known (Z68.-) Z68
  27. E88.89
    Other specified metabolic disorders
    Billable50
    Inclusion terms 1, Excludes1 1

    Inclusion terms

    • Launois-Bensaude adenolipomatosis

    Excludes1

    • adult pulmonary Langerhans cell histiocytosis (J84.82) J84.82
  28. E88.9
    Metabolic disorder, unspecified
    Billable
  29. E88.A
    Wasting disease (syndrome) due to underlying condition
    Billable
    Inclusion terms 1, Excludes1 2, Excludes2 1, Code first 1

    Inclusion terms

    • Cachexia due to underlying condition

    Excludes1

    • cachexia NOS (R64) R64
    • nutritional marasmus (E41) E41

    Excludes2

    Code first

    • underlying condition

CC/MCC: as a secondary diagnosis, unless the principal diagnosis is on the code’s PDX exclusion list (MS-DRG v44 Appendix C; list on the code card).

Sources of each column.

Instructions

At E88

Excludes1

  • histiocytosis X (chronic) (C96.6) C96.6

Use additional code

  • codes for associated conditions

Notes that also apply

Block E70-E88 instructions (Excludes1 5, Excludes2 1): printed on the block page.

Chapter 4 instructions (Notes 1, Excludes1 1): printed on the chapter 4 page.

Index entries

  • Dysplasminogenemia → E88.02
  • Hypoplasminogenemia → E88.02
  • Analbuminemia → E88.09
  • Atransferrinemia, congenital → E88.09
  • Bisalbuminemia → E88.09
  • Dysproteinemia → E88.09
  • Hyperproteinemia → E88.09
  • Hypoalbuminemia → E88.09
  • Para-albuminemia → E88.09
  • Pyroglobulinemia NEC → E88.09
  • Lipodystrophy → E88.10
  • APL (acquired partial lipodystrophy) → E88.11
  • Barraquer (-Simons) disease or syndrome (progressive lipodystrophy) → E88.11
  • Lipodystrophia progressiva → E88.11
  • Simons' disease or syndrome (progressive lipodystrophy) → E88.11
  • AGL (acquired generalized lipodystrophy) → E88.12
  • CGL (congenital generalized lipodystrophy) → E88.12
  • Dercum's disease → E88.2
  • Lipomatosis → E88.2
  • Tumor lysis syndrome (following antineoplastic chemotherapy) (spontaneous) NEC → E88.3
  • MELAS syndrome → E88.41
  • MERRF syndrome (myoclonic epilepsy associated with ragged-red fiber) → E88.42
  • LBSL (leukoencephalopathy with brainstem - spinal cord involvement - lactate elevation) → E88.43
  • LTBL (leukoencephalopathy with thalamus - brainstem involvement - high lactate) → E88.43
  • MNGIE (Mitochondrial Neurogastrointestinal Encephalopathy) syndrome → E88.49
  • NARP (Neuropathy, Ataxia and Retinitis pigmentosa) syndrome → E88.49
  • Dysmetabolic syndrome X → E88.810
  • Metabolic syndrome → E88.810
  • Adenolipomatosis, Launois-Bensaude → E88.89
  • Dihydropyrimidine dehydrogenase disease (DPD) → E88.89
  • DPD (dihydropyrimidine dehydrogenase deficiency) → E88.89
  • Hoffa-Kastert disease → E88.89
  • Hoffa's disease → E88.89
  • Ketosis NEC → E88.89
  • Launois-Bensaude adenolipomatosis → E88.89
  • Liposynovitis prepatellaris → E88.89
  • Steatosis → E88.89
  • Deficiency, deficientalpha-1-antitrypsin → E88.01
  • Deficiency, deficientserum antitrypsin, familial → E88.01
  • Deficiency, deficientplasminogen (type 1) (type 2) → E88.02

82 more, longer entries are not listed; the search box finds them by word.

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