ICD-10 Code E75.243 – Niemann-Pick disease type D (2026): Diagnosis, Symptoms & Billing Guide
2026 ICD-10-CM Diagnosis Code E75.243 – Niemann-Pick disease type D
What it is
E75.243 identifies Niemann-Pick disease type D, a rare inherited lysosomal storage disorder. It is used when the record specifically documents this subtype, rather than another Niemann-Pick form.
Clinical signs
Clinical features vary; refer to documentation. Niemann-Pick disease can involve progressive neurologic decline, hepatosplenomegaly, and other systemic findings, but you should code only what is confirmed in the chart.
When to use this code
Use E75.243 when the provider documents Niemann-Pick disease type D by name or clearly links the diagnosis to this subtype. It may appear in genetic, neurologic, or metabolic evaluations. If the subtype is not stated, check documentation before assigning this code.
Do not use for
Do not use this code for other Niemann-Pick variants, unspecified Niemann-Pick disease, or general lipid storage disorders. Check documentation if the record only says “Niemann-Pick disease” without a type.
Coding tip
Code the exact subtype documented; if the note is vague, query for clarification instead of assuming type D.