ICD-10 Code E75.242 – Niemann-Pick disease type C (2026): Diagnosis, Symptoms & Billing Guide
2026 ICD-10-CM Diagnosis Code E75.242 – Niemann-Pick disease type C
What it is
E75.242 identifies Niemann-Pick disease type C, a rare inherited lipid storage disorder. It is used when the record documents this specific lysosomal transport defect, not another Niemann-Pick type.
Clinical signs
Typical findings include progressive neurologic decline, ataxia, vertical supranuclear gaze palsy, and hepatosplenomegaly. Clinical features vary; refer to documentation for the confirmed phenotype.
When to use this code
Use this code when the provider documents Niemann-Pick disease type C, NPC1/NPC2-related disease, or a confirmed diagnosis of this subtype. It also fits encounters for established disease follow-up or evaluation of complications tied to type C.
If the chart only says Niemann-Pick disease without a type, check documentation before coding. Code the documented subtype rather than assuming type C.
Do not use for
Do not use E75.242 for other Niemann-Pick types, nonspecific Niemann-Pick disease, or suspected disease without confirmation. Check documentation if the subtype is unclear.
Coding tip
Verify the exact subtype in the assessment, genetics note, or problem list before assigning E75.242.