ICD-10 Code E75.23 – Krabbe Disease (2026): Diagnosis, Symptoms & Billing Guide
2026 ICD-10-CM Diagnosis Code E75.23 – Krabbe disease
What it is
E75.23 identifies Krabbe disease, an inherited lysosomal storage disorder that affects myelin in the nervous system. It is also called globoid cell leukodystrophy.
Clinical signs
Clinical features vary; refer to documentation. Common findings include progressive neurologic decline, irritability, feeding difficulty, muscle stiffness, and developmental regression, especially in infants.
When to use this code
Use this code when the provider documents Krabbe disease or globoid cell leukodystrophy as the confirmed diagnosis. It also applies when the record clearly supports this inherited leukodystrophy in a patient being evaluated for progressive neurologic symptoms.
Do not assign it for nonspecific developmental delay, weakness, or suspected metabolic disease without documentation of Krabbe disease. If the chart is unclear, check documentation.
Do not use for
Do not use E75.23 for other leukodystrophies, unspecified metabolic disorders, or symptoms alone. Check documentation if the diagnosis is only suspected.
Coding tip
Code E75.23 only when the provider names Krabbe disease; if the record uses another leukodystrophy term, verify the exact diagnosis first.