ICD-10 Code E75 – Disorders of sphingolipid metabolism and other lipid storage disorders (2026): Diagnosis, Symptoms & Billing Guide
2026 ICD-10-CM Diagnosis Code E75 – Disorders of sphingolipid metabolism and other lipid storage disorders
What it is
E75 identifies inherited metabolic disorders in which sphingolipids or related lipids are not broken down or stored normally. These conditions cause abnormal buildup in tissues and may affect the brain, liver, spleen, bones, or other organs.
Clinical signs
Clinical features vary; refer to documentation. Commonly reported findings include progressive neurologic impairment, organ enlargement, skeletal changes, or developmental delay, depending on the specific disorder within this category.
When to use this code
Use E75 when the provider documents a disorder of sphingolipid metabolism or another lipid storage disorder and does not identify a more specific ICD-10-CM code. It is appropriate for confirmed inherited storage diseases recorded in the assessment or problem list. If the exact condition is named, code that specific diagnosis instead.
Do not use for
Do not use E75 for acquired lipid disorders, routine hyperlipidemia, or nonspecific abnormal lipid findings. If documentation points to a different metabolic disease or a more specific storage disorder, choose the more precise code.
Coding tip
Check the chart for the exact named disorder; E75 is a category code, so specificity in the provider’s documentation matters.