ICD-10 Code A39.1 – Waterhouse-Friderichsen syndrome (2026): Diagnosis, Symptoms & Billing Guide
2026 ICD-10-CM Diagnosis Code A39.1 – Waterhouse-Friderichsen syndrome
What it is
Waterhouse-Friderichsen syndrome is a severe meningococcal infection complication marked by sudden adrenal hemorrhage and acute adrenal failure. It is a medical emergency and usually reflects overwhelming sepsis.
Clinical signs
Clinical features vary; refer to documentation. Typical findings include rapid deterioration, shock, purpura or petechiae, and evidence of adrenal hemorrhage or insufficiency in the setting of invasive meningococcal disease.
When to use this code
Use A39.1 when the record specifically documents Waterhouse-Friderichsen syndrome or meningococcal sepsis with adrenal hemorrhage. Apply it when the provider links the syndrome to the infection and not just to nonspecific sepsis or rash. Check documentation if the diagnosis is uncertain.
Do not use for
Do not use this code for meningococcal infection without adrenal hemorrhage or for adrenal insufficiency from another cause. Do not assign it when the chart only mentions sepsis, shock, or purpura without the syndrome being documented.
Coding tip
Confirm that the provider explicitly documents Waterhouse-Friderichsen syndrome before assigning A39.1.