Other and unspecified metabolic disorders (E88) ICD-10-CM
The E88 code range covers other and unspecified metabolic disorders with 30 ICD-10-CM diagnosis codes. 24 of them are billable and valid for claim submission in fiscal year 2027, and the category headers group them but cannot themselves be billed.
Use Additional Code
The “use additional code” indicates that a secondary code could be used to further specify the patient’s condition. This note is not mandatory and is only used if enough information is available to assign an additional code.
- codes for associated conditions
Type 1 Excludes
A type 1 excludes note is a pure excludes note. It means "NOT CODED HERE!" An Excludes1 note indicates that the code excluded should never be used at the same time as the code above the Excludes1 note. An Excludes1 is used when two conditions cannot occur together, such as a congenital form versus an acquired form of the same condition.
- histiocytosis X chronic C96.6
Codes in the E88 Range 30 codes · 24 billable
- E88 Other and unspecified metabolic disordersNon-billable
- E88.0 Disorders of plasma-protein metabolism, not elsewhere classifiedNon-billable
- 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 classifiedNon-billable
- E88.10 Lipodystrophy, unspecified
- E88.11 Partial lipodystrophy
- E88.12 Generalized lipodystrophy
- E88.13 Localized lipodystrophy
- E88.14 HIV-associated lipodystrophy
- E88.19 Other lipodystrophy, not elsewhere classified
- E88.2 Lipomatosis, not elsewhere classified
- E88.3 Tumor lysis syndrome
- E88.4 Mitochondrial metabolism disordersNon-billable
- E88.40 Mitochondrial metabolism disorder, unspecified
- E88.41 MELAS syndrome
- E88.42 MERRF syndrome
- E88.43 Disorders of mitochondrial tRNA synthetases
- E88.49 Other mitochondrial metabolism disorders
- E88.8 Other specified metabolic disordersNon-billable
- E88.81 Metabolic syndrome and other insulin resistanceNon-billable
- E88.810 Metabolic syndrome
- E88.811 Insulin resistance syndrome, Type A
- E88.818 Other insulin resistance
- E88.819 Insulin resistance, unspecified
- E88.82 Obesity due to disruption of MC4R pathway
- E88.89 Other specified metabolic disorders
- E88.9 Metabolic disorder, unspecified
- E88.A Wasting disease (syndrome) due to underlying condition
Clinical Terms in This Code Range
Definitions from the National Library of Medicine for conditions coded in the E88 range.
alpha 1-Antitrypsin Deficiency
Deficiency of the protease inhibitor ALPHA 1-ANTITRYPSIN that manifests primarily as PULMONARY EMPHYSEMA and LIVER CIRRHOSIS.
Focal Lipodystrophy
Loss of subcutaneous fat confined to small area(s) of the body.
HIV-Associated Lipodystrophy Syndrome
Defective metabolism leading to fat maldistribution in patients infected with HIV. The etiology appears to be multifactorial and probably involves some combination of infection-induced alterations in metabolism, direct effects of antiretroviral therapy, and patient-related factors.
Hypoalbuminemia
A condition in which albumin level in blood (SERUM ALBUMIN) is below the normal range. Hypoalbuminemia may be due to decreased hepatic albumin synthesis, increased albumin catabolism, altered albumin distribution, or albumin loss through the urine (ALBUMINURIA).
Insulin Resistance
Diminished effectiveness of INSULIN in lowering blood sugar levels: requiring the use of 200 units or more of insulin per day to prevent HYPERGLYCEMIA or KETOSIS.
Insulin Resistant Diabetes Mellitus with Acanthosis Nigricans and Hyperandrogenism
A syndrome of insulin resistance caused by mutation(s) in the INSR gene, encoding the insulin receptor. This condition is characterized by a clinical triad of hyperinsulinemia, acanthosis nigricans, and hyperandrogenism without lipodystrophy. This is the least severe of a spectrum of disorders; the other two conditions are Rabson-Mendenhall syndrome and Donohoe syndrome.
Lipodystrophy
A collection of heterogenous conditions resulting from defective LIPID METABOLISM and characterized by ADIPOSE TISSUE atrophy. Often there is redistribution of body fat resulting in peripheral fat wasting and central adiposity. They include generalized, localized, congenital, and acquired lipodystrophy.
Lipodystrophy, Congenital Generalized
Congenital disorders, usually autosomal recessive, characterized by severe generalized lack of ADIPOSE TISSUE, extreme INSULIN RESISTANCE, and HYPERTRIGLYCERIDEMIA.
Lipomatosis
A disorder characterized by the accumulation of encapsulated or unencapsulated tumor-like fatty tissue resembling LIPOMA.
MELAS Syndrome
A mitochondrial disorder characterized by focal or generalized seizures, episodes of transient or persistent neurologic dysfunction resembling strokes, and ragged-red fibers on muscle biopsy. Affected individuals tend to be normal at birth through early childhood, then experience growth failure, episodic vomiting, and recurrent cerebral insults resulting in visual loss and hemiparesis. The cortical lesions tend to occur in the parietal and occipital lobes and are not associated with vascular occlusion. VASCULAR HEADACHE is frequently associated and the disorder tends to be familial. (From Joynt, Clinical Neurology, 1992, Ch56, p117)
MERRF Syndrome
A mitochondrial encephalomyopathy characterized clinically by a mixed seizure disorder, myoclonus, progressive ataxia, spasticity, and a mild myopathy. Dysarthria, optic atrophy, growth retardation, deafness, and dementia may also occur. This condition tends to present in childhood and to be transmitted via maternal lineage. Muscle biopsies reveal ragged-red fibers and respiratory chain enzymatic defects. (From Adams et al., Principles of Neurology, 6th ed, p986)
Metabolic Disorder
A congenital (due to inherited enzyme abnormality) or acquired (due to failure of a metabolic important organ) disorder resulting from an abnormal metabolic process.
Metabolic Syndrome
A cluster of symptoms that are risk factors for CARDIOVASCULAR DISEASES and TYPE 2 DIABETES MELLITUS. The major components of metabolic syndrome include ABDOMINAL OBESITY; atherogenic DYSLIPIDEMIA; HYPERTENSION; HYPERGLYCEMIA; INSULIN RESISTANCE; a proinflammatory state; and a prothrombotic (THROMBOSIS) state.
Partial Lipodystrophy
Loss and redistribution of subcutaneous and/or visceral adipose tissue from specific regions of the body.
Tumor Lysis Syndrome
A syndrome resulting from cytotoxic therapy, occurring generally in aggressive, rapidly proliferating lymphoproliferative disorders. It is characterized by combinations of hyperuricemia, lactic acidosis, hyperkalemia, hyperphosphatemia and hypocalcemia.
About the E88 Code Range
Metabolic disorders affect how the body processes substances. This category groups other specified disorders with those whose type is unspecified.
The subdivisions separate plasma-protein metabolism disorders under E88.0, lipodystrophy under E88.1, and mitochondrial metabolism disorders under E88.4. Each has more specific subdivisions. Separate codes identify lipomatosis, tumor lysis syndrome, and wasting disease due to an underlying condition.
E88.8 includes metabolic syndrome, insulin resistance, and obesity due to disruption of the MC4R pathway. E88.9 identifies an unspecified metabolic disorder.
FY 2027 changes: The FY 2027 ICD-10-CM update, effective October 1, 2026, deleted E88.1.
Questions About This Page
How many billable codes are in the E88 range?
Of the 30 codes in this range, 24 are billable and valid for claim submission from October 1, 2026 through September 30, 2027. Category header codes group them but cannot be reported on claims.
What does the E88 range classify?
The range classifies other and unspecified metabolic disorders. Each code links to its own reference page with billing status, MS-DRG grouping, coding notes, and clinical information.
Related References
Source: CMS FY 2027 ICD-10-CM Tabular List and order file, effective October 1, 2026 through September 30, 2027.
