Disorders of lipoprotein metabolism and other lipidemias (E78) ICD-10-CM
The E78 code range covers disorders of lipoprotein metabolism and other lipidemias with 24 ICD-10-CM diagnosis codes. 18 of them are billable and valid for claim submission in fiscal year 2027, and the category headers group them but cannot themselves be billed.
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.
Codes in the E78 Range 24 codes · 18 billable
- E78 Disorders of lipoprotein metabolism and other lipidemiasNon-billable
- E78.0 Pure hypercholesterolemiaNon-billable
- E78.00 Pure hypercholesterolemia, unspecified
- E78.01 Familial hypercholesterolemiaNon-billable
- E78.010 Homozygous familial hypercholesterolemia [HoFH]
- E78.011 Heterozygous familial hypercholesterolemia [HeFH]
- E78.019 Familial hypercholesterolemia, unspecified
- E78.1 Pure hyperglyceridemia
- E78.2 Mixed hyperlipidemia
- E78.3 Hyperchylomicronemia
- E78.4 Other hyperlipidemiaNon-billable
- E78.41 Elevated Lipoprotein(a)
- E78.49 Other hyperlipidemia
- E78.5 Hyperlipidemia, unspecified
- E78.6 Lipoprotein deficiency
- E78.7 Disorders of bile acid and cholesterol metabolismNon-billable
- E78.70 Disorder of bile acid and cholesterol metabolism, unspecified
- E78.71 Barth syndrome
- E78.72 Smith-Lemli-Opitz syndrome
- E78.79 Other disorders of bile acid and cholesterol metabolism
- E78.8 Other disorders of lipoprotein metabolismNon-billable
- E78.81 Lipoid dermatoarthritis
- E78.89 Other lipoprotein metabolism disorders
- E78.9 Disorder of lipoprotein metabolism, unspecified
Clinical Terms in This Code Range
Definitions from the National Library of Medicine for conditions coded in the E78 range.
Abetalipoproteinemia
An autosomal recessive disorder of lipid metabolism. It is caused by mutation of the microsomal triglyceride transfer protein that catalyzes the transport of lipids (TRIGLYCERIDES; CHOLESTEROL ESTERS; PHOSPHOLIPIDS) and is required in the secretion of BETA-LIPOPROTEINS (low density lipoproteins or LDL). Features include defective intestinal lipid absorption, very low serum cholesterol level, and near absent LDL.
Barth Syndrome
Rare congenital X-linked disorder of lipid metabolism. Barth syndrome is transmitted in an X-linked recessive pattern. The syndrome is characterized by muscular weakness, growth retardation, DILATED CARDIOMYOPATHY, variable NEUTROPENIA, 3-methylglutaconic aciduria (type II) and decreases in mitochondrial CARDIOLIPIN level. Other biochemical and morphological mitochondrial abnormalities also exist.
Homozygous Familial Hypercholesterolemia
A rare inherited genetic disorder, one form of HYPERLIPOPROTEINEMIA TYPE II, characterized by high level of LOW-DENSITY LIPOPROTEIN (LDL) which if not treated could elevate the chance of heart attack at an early age.
Hypercholesterolemia
A condition with abnormally high levels of CHOLESTEROL in the blood. It is defined as a cholesterol value exceeding the 95th percentile for the population.
Hyperlipidemias
Conditions with excess LIPIDS in the blood.
Hyperlipoproteinemia Type II
A group of familial disorders characterized by elevated circulating cholesterol contained in either LOW-DENSITY LIPOPROTEINS alone or also in VERY-LOW-DENSITY LIPOPROTEINS (pre-beta lipoproteins).
Hyperlipoproteinemia Type III
An autosomal recessively inherited disorder characterized by the accumulation of intermediate-density lipoprotein (IDL or broad-beta-lipoprotein). IDL has a CHOLESTEROL to TRIGLYCERIDES ratio greater than that of VERY-LOW-DENSITY LIPOPROTEINS. This disorder is due to mutation of APOLIPOPROTEINS E, a receptor-binding component of VLDL and CHYLOMICRONS, resulting in their reduced clearance and high plasma levels of both cholesterol and triglycerides.
Hyperlipoproteinemia Type IV
A hypertriglyceridemia disorder, often with autosomal dominant inheritance. It is characterized by the persistent elevations of plasma TRIGLYCERIDES, endogenously synthesized and contained predominantly in VERY-LOW-DENSITY LIPOPROTEINS (pre-beta lipoproteins). In contrast, the plasma CHOLESTEROL and PHOSPHOLIPIDS usually remain within normal limits.
Hypertriglyceridemia
A condition of elevated levels of TRIGLYCERIDES in the blood.
Hypoalphalipoproteinemias
Conditions with abnormally low levels of ALPHA-LIPOPROTEINS (high-density lipoproteins) in the blood. Hypoalphalipoproteinemia can be associated with mutations in genes encoding APOLIPOPROTEIN A-I; LECITHIN CHOLESTEROL ACYLTRANSFERASE; and ATP-BINDING CASSETTE TRANSPORTERS.
Hypobetalipoproteinemias
Conditions with abnormally low levels of BETA-LIPOPROTEINS (low density lipoproteins or LDL) in the blood. It is defined as LDL values equal to or less than the 5th percentile for the population. They include the autosomal dominant form involving mutation of the APOLIPOPROTEINS B gene, and the autosomal recessive form involving mutation of the microsomal triglyceride transfer protein. All are characterized by low LDL and dietary fat malabsorption.
Hypolipoproteinemias
Conditions with abnormally low levels of LIPOPROTEINS in the blood. This may involve any of the lipoprotein subclasses, including ALPHA-LIPOPROTEINS (high-density lipoproteins); BETA-LIPOPROTEINS (low-density lipoproteins); and PREBETA-LIPOPROTEINS (very-low-density lipoproteins).
Lipoid Proteinosis of Urbach and Wiethe
An autosomal recessive disorder characterized by glassy degenerative thickening (hyalinosis) of SKIN; MUCOSA; and certain VISCERA. This disorder is caused by mutation in the extracellular matrix protein 1 gene (ECM1). Clinical features include hoarseness and skin eruption due to widespread deposition of HYALIN.
Smith-Lemli-Opitz Syndrome
An autosomal recessive disorder of CHOLESTEROL metabolism. It is caused by a deficiency of 7-dehydrocholesterol reductase, the enzyme that converts 7-dehydrocholesterol to cholesterol, leading to an abnormally low plasma cholesterol. This syndrome is characterized by multiple CONGENITAL ABNORMALITIES, growth deficiency, and INTELLECTUAL DISABILITY.
Tangier Disease
An autosomal recessively inherited disorder caused by mutation of ATP-BINDING CASSETTE TRANSPORTERS involved in cellular cholesterol removal (reverse-cholesterol transport). It is characterized by near absence of ALPHA-LIPOPROTEINS (high-density lipoproteins) in blood. The massive tissue deposition of cholesterol esters results in HEPATOMEGALY; SPLENOMEGALY; RETINITIS PIGMENTOSA; large orange tonsils; and often sensory POLYNEUROPATHY. The disorder was first found among inhabitants of Tangier Island in the Chesapeake Bay, MD.
About the E78 Code Range
These disorders involve lipoproteins, blood fats, bile acids, or cholesterol. The categories distinguish cholesterol alone, other specific fat disorders, and disorders without a specified type.
E78.0 covers pure hypercholesterolemia, or high cholesterol, with a familial branch divided by subtype. E78.1 identifies pure hyperglyceridemia, while E78.2 identifies mixed hyperlipidemia, involving more than one type of blood fat. Other branches distinguish hyperchylomicronemia, elevated lipoprotein(a), lipoprotein deficiency, and unspecified hyperlipidemia.
E78.7 separates bile acid and cholesterol metabolism disorders by named syndrome, other type, or unspecified type. E78.8 identifies other lipoprotein metabolism disorders; E78.9 leaves the disorder unspecified.
FY 2027 changes: The FY 2027 ICD-10-CM update, effective October 1, 2026, deleted E78.01.
Questions About This Page
How many billable codes are in the E78 range?
Of the 24 codes in this range, 18 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 E78 range classify?
The range classifies disorders of lipoprotein metabolism and other lipidemias. 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.
