ICD-10 Code E75.248 – Other Niemann-Pick disease (2026): Diagnosis, Symptoms & Billing Guide

The ICD-10 code for Other Niemann-Pick disease is E75.248.
2026 ICD-10-CM Diagnosis Code E75.248 – Other Niemann-Pick disease

What it is

E75.248 identifies a form of Niemann-Pick disease not classified elsewhere. Use it for documented sphingolipid storage disorder cases when the record specifies “other” Niemann-Pick disease.

Clinical signs

Clinical features vary; refer to documentation. Commonly reported findings in Niemann-Pick disorders include organ enlargement, neurologic involvement, and progressive multisystem disease.

When to use this code

Use this code when the provider documents other Niemann-Pick disease and does not name a more specific subtype. It may also fit when the chart confirms a Niemann-Pick disorder but the exact variant is not stated. Check documentation for the most specific diagnosis available.

Do not use for

Do not use it for a clearly identified Niemann-Pick subtype with its own code. Avoid assigning it when the diagnosis is only suspected or when documentation supports another lysosomal storage disorder.

Coding tip

Query the provider if the subtype is unclear, because specificity can change the correct ICD-10 code.

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