ICD-10 Code E70.330 – Chediak-Higashi Syndrome (2026): Diagnosis, Symptoms & Billing Guide
2026 ICD-10-CM Diagnosis Code E70.330 – Chediak-Higashi syndrome
What it is
Chediak-Higashi syndrome is a rare inherited disorder that affects immune function and pigmentation. It is caused by abnormal lysosomal trafficking, leading to problems with white blood cells and other body cells.
Clinical signs
Typical findings include recurrent pyogenic infections, partial albinism, and neurologic abnormalities. Clinical features vary; refer to documentation.
When to use this code
Use E70.330 when the provider documents Chediak-Higashi syndrome by name or clearly identifies the inherited condition. This code applies to confirmed diagnoses and problem list entries when the condition is relevant to care. Check documentation if the record only mentions immune deficiency or albinism without the syndrome name.
Do not use for
Do not use this code for isolated albinism, generic immunodeficiency, or recurrent infection without documentation of Chediak-Higashi syndrome. If the diagnosis is uncertain, check documentation.
Coding tip
Code the syndrome only when the provider links the findings to Chediak-Higashi syndrome, not when features are listed separately.