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ICD-10/E78.00

E78.00 ICD-10-CM Code: Pure hypercholesterolemia, unspecified

E78.00 is the authoritative medical code for Pure hypercholesterolemia, unspecified. This classification is used in medical billing and clinical recording to specify the clinical criteria for pure hypercholesterolemia, unspecified (ICD-10-CM E78.00), ensuring healthcare documentation aligns with current federal coding standards.

Billing Status: YES. This is a valid, specific, and billable ICD-10-CM reference.

Inpatient Payment Groups (MS-DRG)

MS-DRGs this diagnosis helps define, as principal or secondary, per the CMS ICD-10-CM/PCS MS-DRG Definitions Manual v43.0 (FY2026), Appendix B.

  • MS-DRG 642 — INBORN AND OTHER DISORDERS OF METABOLISM (MDC 10)

A diagnosis appearing in a group's logic does not by itself determine the DRG assigned to a stay; the grouper uses the full claim.

Code Set: Valid FY2026 and FY2027. FY2026 took effect October 1, 2025.

Official Registry Overview & Definition

Pure hypercholesterolemia, unspecified is a billable ICD-10-CM diagnosis code (E78.00). Inclusion terms: Fredrickson's hyperlipoproteinemia, type IIa; Hyperbetalipoproteinemia; Low-density-lipoprotein-type [LDL] hyperlipoproteinemia; (Pure) hypercholesterolemia NOS. Excludes1 (not coded here): sphingolipidosis (E75.0-E75.3); androgen insensitivity syndrome (E34.5-); congenital adrenal hyperplasia (E25.0); hemolytic anemias attributable to enzyme disorders (D55.-); Marfan syndrome (Q87.4-); 5-alpha-reductase deficiency (E29.1). Excludes2 (not included here): Ehlers-Danlos syndromes (Q79.6-).

Official Tabular Instructional Notes

Sequencing, inclusion, and exclusion notes published for E78.00 in the official ICD-10-CM tabular list.

Inclusion Terms

Alternative terms the tabular list files under this code.

  • Fredrickson's hyperlipoproteinemia, type IIa
  • Hyperbetalipoproteinemia
  • Low-density-lipoprotein-type [LDL] hyperlipoproteinemia
  • (Pure) hypercholesterolemia NOS

Excludes1 — Not Coded Here

Conditions that can never be reported with this code; the two are mutually exclusive.

  • sphingolipidosis (E75.0-E75.3)
  • androgen insensitivity syndrome (E34.5-)
  • congenital adrenal hyperplasia (E25.0)
  • hemolytic anemias attributable to enzyme disorders (D55.-)
  • Marfan syndrome (Q87.4-)
  • 5-alpha-reductase deficiency (E29.1)

Excludes2 — Not Included Here

Conditions not covered by this code, but which may be reported alongside it when both are present.

  • Ehlers-Danlos syndromes (Q79.6-)

Frequently Asked Questions (FAQ) & Clinical Guidance

What can't be coded together with E78.00?

Per official ICD-10-CM Excludes1 instructions, E78.00 must not be reported together with: sphingolipidosis (E75.0-E75.3); androgen insensitivity syndrome (E34.5-); congenital adrenal hyperplasia (E25.0); hemolytic anemias attributable to enzyme disorders (D55.-).

Can E78.00 be reported alongside related conditions?

Per official ICD-10-CM Excludes2 instructions, E78.00 and the following are not considered part of each other and may both be reported when both conditions are present: Ehlers-Danlos syndromes (Q79.6-).

What conditions are included under E78.00?

Pure hypercholesterolemia, unspecified includes: Fredrickson's hyperlipoproteinemia, type IIa; Hyperbetalipoproteinemia; Low-density-lipoprotein-type [LDL] hyperlipoproteinemia; (Pure) hypercholesterolemia NOS.

Nearest Codes in This Family

Official ICD-10-CM classifications closest to E78.00 in its code family, with their registry titles.

  • E78 — Disorders of lipoprotein metabolism and other lipidemias
  • E78.0 — Pure hypercholesterolemia
  • E78.01 — Familial hypercholesterolemia
  • 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 hyperlipidemia

View all codes in the E78 family

Indexed Clinical Terms (15)

Clinical term phrases from the official ICD-10-CM Index to Diseases and Injuries that map to this code.

  • Cholesteremia
  • Cholesterol, elevated (high)
  • Cholesterolemia (essential) (pure)
  • Elevated, elevation, cholesterol
  • Findings, abnormal, inconclusive, without diagnosis, cholesterol, high
  • Frederickson's hyperlipoproteinemia, type, IIA
  • High, cholesterol
  • Hyperbetalipoproteinemia (familial)
  • Hypercholesterolemia (essential) (primary) (pure)
  • Hyperlipemia, hyperlipidemia, group, A
  • Hyperlipoproteinemia, Fredrickson's type, IIa
  • Hyperlipoproteinemia, low-density-lipoprotein-type (LDL)
  • Low-density-lipoprotein-typehyperlipoproteinemia (LDL)
  • Xanthoma (s), xanthomatosis (primary) (familial) (hereditary), hypercholesterinemic
  • Xanthoma (s), xanthomatosis (primary) (familial) (hereditary), hypercholesterolemic