ICD-10 Code E78.010 – Homozygous familial hypercholesterolemia [HoFH] (2026): Diagnosis, Symptoms & Billing Guide
2026 ICD-10-CM Diagnosis Code E78.010 – Homozygous familial hypercholesterolemia [HoFH]
What it is
E78.010 identifies homozygous familial hypercholesterolemia, a rare inherited disorder causing very high LDL cholesterol from birth. It reflects a severe genetic form of hypercholesterolemia, not ordinary diet-related high cholesterol.
Clinical signs
Clinical features may include markedly elevated cholesterol, early-onset atherosclerotic disease, and tendon or cutaneous xanthomas. Findings vary; refer to documentation for genetic confirmation or specialist assessment.
When to use this code
Use this code when the record specifically documents homozygous familial hypercholesterolemia or HoFH. It may also apply when the clinician confirms the diagnosis based on genetic testing, family history, and characteristic lipid abnormalities.
Choose this code for encounters focused on managing or evaluating the disorder, even if the patient is asymptomatic at that visit. If documentation only says “familial hypercholesterolemia” without homozygous status, check documentation before assigning E78.010.
Do not use for
Do not use this code for general hyperlipidemia, mixed dyslipidemia, or heterozygous familial hypercholesterolemia. If the record does not clearly state HoFH, check documentation.
Coding tip
Assign E78.010 only when the chart explicitly supports homozygous familial hypercholesterolemia; otherwise, verify the exact inherited lipid disorder documented.