ICD-10 Code E75.3 – Sphingolipidosis, unspecified (2026): Diagnosis, Symptoms & Billing Guide
2026 ICD-10-CM Diagnosis Code E75.3 – Sphingolipidosis, unspecified
What it is
E75.3 identifies an unspecified sphingolipidosis, a group of inherited disorders caused by abnormal sphingolipid breakdown and storage. Use it when the record names sphingolipidosis but does not specify the exact type.
Clinical signs
Clinical features vary; refer to documentation. Commonly reported findings in sphingolipidoses include neurologic decline, organ enlargement, or developmental delay, but the exact presentation depends on the specific disorder.
When to use this code
Use this code when the clinician documents sphingolipidosis without naming a more specific diagnosis. It may also fit when the chart confirms a sphingolipid storage disorder, but the subtype is not available in the available record.
Do not guess the subtype from symptoms alone. If later documentation identifies a specific sphingolipidosis, code that condition instead of E75.3.
Do not use for
Do not use E75.3 for a clearly documented specific sphingolipidosis such as a named lysosomal storage disorder. Also avoid it when the record only suggests a disorder and the diagnosis is not confirmed; check documentation.
Coding tip
Query the provider when the note says “sphingolipidosis” but omits the exact type, because specificity can change the final ICD-10-CM code.