ICD-10 Code E71.53 – Other group 2 peroxisomal disorders (2026): Diagnosis, Symptoms & Billing Guide
2026 ICD-10-CM Diagnosis Code E71.53 – Other group 2 peroxisomal disorders
What it is
E71.53 identifies other group 2 peroxisomal disorders, a category of inherited metabolic conditions caused by abnormal peroxisome function. These disorders affect how the body breaks down certain fatty acids and related substances.
Clinical signs
Clinical features vary; refer to documentation. Standard references describe neurologic, developmental, hepatic, or ocular findings in peroxisomal disease, but the exact pattern depends on the specific disorder.
When to use this code
Use this code when the record documents a diagnosed group 2 peroxisomal disorder that is not better described by a more specific ICD-10-CM code. It is appropriate for confirmed inherited peroxisomal disease and for encounters focused on that diagnosis. If the chart names a specific syndrome, code that condition instead.
Do not use for
Do not use E71.53 for nonspecific metabolic workups, suspected disease, or symptoms alone. Check documentation if the provider identifies a different peroxisomal disorder or another metabolic condition.
Coding tip
Code the most specific documented peroxisomal diagnosis first, and use E71.53 only when no more specific code applies.