ICD-10 Code E71.5 – Peroxisomal disorders (2026): Diagnosis, Symptoms & Billing Guide
2026 ICD-10-CM Diagnosis Code E71.5 – Peroxisomal disorders
What it is
E71.5 identifies inherited disorders caused by abnormal peroxisome function. These conditions disrupt normal metabolism of very-long-chain fatty acids and related substances.
Clinical signs
Clinical features vary; refer to documentation. Common findings may include developmental delay, neurologic impairment, vision or hearing problems, and liver involvement in recognized peroxisomal disease.
When to use this code
Use E71.5 when the provider documents a peroxisomal disorder, such as a confirmed inherited peroxisome biogenesis or single-enzyme defect. Code it from the documented diagnosis, not from isolated abnormal screening results. If the record only says “possible” or “rule out,” check documentation before assigning the code.
Do not use for
Do not use this code for nonspecific metabolic abnormalities without a documented peroxisomal disorder. Also avoid it when the chart names a different inherited condition that is not peroxisomal.
Coding tip
Match the code to the exact disorder named in the record and query if the type of peroxisomal disease is unclear.