ICD-10 Code E75.21 – Fabry (-Anderson) disease (2026): Diagnosis, Symptoms & Billing Guide
2026 ICD-10-CM Diagnosis Code E75.21 – Fabry (-Anderson) disease
What it is
E75.21 identifies Fabry (-Anderson) disease, an inherited lysosomal storage disorder. It causes abnormal buildup of a fatty substance in multiple organs and is documented as a specific metabolic diagnosis.
Clinical signs
Clinical features vary; refer to documentation. Commonly described findings include neuropathic pain, reduced sweating, skin lesions, kidney involvement, cardiac disease, and stroke-like events.
When to use this code
Use E75.21 when the provider documents Fabry disease, Fabry-Anderson disease, or a confirmed diagnosis of this specific disorder. Code it from the medical record when the condition is the reason for evaluation, monitoring, or ongoing care.
If the chart only mentions family history, carrier status, or a suspected metabolic disorder, do not assign this code without confirmation. Check documentation when the exact diagnosis is unclear.
Do not use for
Do not use E75.21 for nonspecific lipid disorders, other storage diseases, or symptoms alone. Do not apply it when Fabry disease is only suspected or listed as ruled out.
Coding tip
Confirm the provider’s exact wording, because Fabry disease may be documented under different names, but the code is the same.