ICD-10 Code E75.11 – Mucolipidosis IV (2026): Diagnosis, Symptoms & Billing Guide
2026 ICD-10-CM Diagnosis Code E75.11 – Mucolipidosis IV
What it is
E75.11 identifies mucolipidosis IV, a rare inherited lysosomal storage disorder. It is used when documentation confirms this specific diagnosis.
Clinical signs
Clinical features vary; refer to documentation. Commonly reported findings include developmental delay, vision problems, and progressive neurologic impairment.
When to use this code
Use E75.11 when the provider documents mucolipidosis IV by name, or when the record clearly states this exact disorder. It may appear in genetic, neurologic, or metabolic evaluations. If the note only says “lysosomal storage disease,” check documentation before assigning this code.
Do not use for
Do not use this code for other mucolipidoses, nonspecific metabolic disorders, or suspected disease without confirmation. Check documentation if the diagnosis is incomplete or uncertain.
Coding tip
Code the most specific documented lysosomal storage diagnosis; if the chart only lists a broader category, query for clarification.