ICD-10 Code E75.249 – Niemann-Pick disease, unspecified (2026): Diagnosis, Symptoms & Billing Guide
2026 ICD-10-CM Diagnosis Code E75.249 – Niemann-Pick disease, unspecified
What it is
E75.249 identifies Niemann-Pick disease when the specific type is not documented. Use it for a lysosomal storage disorder involving abnormal lipid accumulation, but only when the record does not name a more specific subtype.
Clinical signs
Clinical features vary; refer to documentation. Standard references describe hepatosplenomegaly, neurologic decline, and developmental delay in some forms, but the presentation depends on the Niemann-Pick subtype.
When to use this code
Use this code when the clinician documents Niemann-Pick disease without specifying type A, B, or C, and the chart does not support a more precise code. It also fits problem lists or discharge summaries that state the disorder generically. If the subtype is identified later, code the documented type instead.
Do not use for
Do not use this code when the record clearly identifies a specific Niemann-Pick type or another lipid storage disorder. Check documentation if the chart only mentions suspected disease or family history.
Coding tip
Query the provider for the subtype whenever the note says only “Niemann-Pick disease” and the type can be clarified.