2026 ICD-10-CM Diagnosis Code E78.00Pure hypercholesterolemia, unspecified

ICD-10-CM Codes›E00–E89›E70-E88›E78

ICD-10-CM E78.00
CMSSource: CMS FY 2026 ICD-10-CM dataset · Effective Oct 1, 2025 – Sep 30, 2026

E78.00 is a billable ICD-10-CM diagnosis code for pure hypercholesterolemia, unspecified. It is valid on HIPAA claims for fiscal year 2026 (October 1, 2025 through September 30, 2026). In AHRQ's Clinical Classifications Software (CCSR), this diagnosis falls under Disorders of lipid metabolism.

Code Identity

ICD-10-CM Code
E78.00
Billable Status
Yes — Valid for Submission
Code Describes
Pure hypercholesterolemia, unspecified
Short Description
Pure hypercholesterolemia, unspecified
Same as the full description in the CMS dataset.
Parent Code
E78.0
Pure hypercholesterolemia

Code Classification

ChapterE00–E89Endocrine, nutritional and metabolic diseases
SectionE70-E88Metabolic disorders
CategoryE78Disorders of lipoprotein metabolism and other lipidemias
This CodeE78.00Pure hypercholesterolemia, unspecified

Approximate SynonymsGuidance

Alternate terms and clinical phrases that map to this code.

  • Familial hypercholesterolemia
  • Familial hyperlipoproteinemia
  • Fredrickson type IIa hyperlipoproteinemia
  • Hyperalphalipoproteinemia
  • Hyperbetalipoproteinemia
  • Hypercholesterolemia
  • Hypercholesterolemia well controlled
  • Hyperlipidemia, group A
  • Polygenic hypercholesterolemia
  • Primary hypercholesterolemia
  • Pure hypercholesterolemia
  • Serum cholesterol above reference range
  • Xanthoma due to abnormality of lipid metabolism
  • Xanthoma due to primary hypercholesterolemia

Tabular List NotesGuidance

Coding notes and annotation back-references applicable to this code.

Inclusion Terms

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

Index to Diseases and InjuriesGuidance

Alphabetical index entries that point to this code.

External Cause of Injuries IndexGuidance

References for this code in the External Cause of Injuries Index.

    • 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-type(LDL) hyperlipoproteinemia
    • Xanthoma(s), xanthomatosis (primary) (familial) (hereditary)
      • hypercholesterinemic
    • Xanthoma(s), xanthomatosis (primary) (familial) (hereditary)
      • hypercholesterolemic

Clinical ClassificationClinical

AHRQ’s CCSR groups this code into broader clinical categories.

CCSR END010
Disorders of lipid metabolism
Default principal diagnosis: inpatient Yes · outpatient Yes

Clinical InformationClinical

  • 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.

  • 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.

  • Cholesterol

    the principal sterol of all higher animals, distributed in body tissues, especially the brain and spinal cord, and in animal fats and oils.

Patient EducationClinical

Cholesterol

Cholesterol is a waxy, fat-like substance that's found in all the cells in your body. Your body needs some cholesterol to make hormones, vitamin D, and substances that help you digest foods. Your body makes all the cholesterol it needs. Cholesterol is also found in foods from animal sources, such as egg yolks, meat, and cheese.

The full article covers:

  • What is cholesterol?
  • What are HDL, LDL, and VLDL?
  • What causes high cholesterol?
  • What can raise my risk of high cholesterol?
  • What other health problems can high cholesterol cause?
  • How is high cholesterol diagnosed?
  • How can I lower my cholesterol?

Read the full article at MedlinePlus

Courtesy of MedlinePlus, a service of the U.S. National Library of Medicine.

Convert E78.00 to ICD-9-CMHistory

The closest ICD-9-CM equivalents under the General Equivalence Mappings.

ICD-9-CM
272.0 Pure hypercholesterolem
Approximate The match is approximate rather than exact.

Code History & ChangesHistory

Replacement E78.00 replaces the following previously assigned code(s):

  • E78.0 - Pure hypercholesterolemia
FY 2018AddedAdded to the ICD-10-CM code setEffective October 1, 2017.
FY 2019–2025No changes
FY 2026CurrentCurrent code set, no changesEffective October 1, 2025 through September 30, 2026.

Questions About E78.00Overview

Is E78.00 (Pure hypercholesterolemia) a billable code?

Yes. This is a billable ICD-10-CM code, specific enough to report pure hypercholesterolemia, unspecified on HIPAA-covered claims from October 1, 2025 through September 30, 2026.

What is the ICD-9 equivalent of E78.00?

Under the General Equivalence Mappings, pure hypercholesterolemia, unspecified converts to ICD-9-CM 272.0 (pure hypercholesterolem). The mapping is approximate, so confirm the match fits the documentation.

Footnotes

[1] Chronic - a chronic condition code indicates a condition lasting 12 months or longer and its effect on the patient based on one or both of the following criteria:

  • The condition results in the need for ongoing intervention with medical products,treatment, services, and special equipment
  • The condition places limitations on self-care, independent living, and social interactions.