Waterhouse-Friderichsen syndrome

Waterhouse-Friderichsen syndrome is a life-threatening condition characterized by acute bilateral adrenal hemorrhage leading to primary adrenal insufficiency. The pathophysiology involves disseminated intravascular coagulation (DIC) and endotoxic shock, typically from Neisseria meningitidis infection, causing hemorrhage into both adrenal glands. This results in complete loss of cortisol and aldosterone production. Clinical presentation includes rapid onset of shock, fever, petechial/purpuric rash (particularly non-blanching purpura), hypotension, and signs of adrenal crisis (nausea, vomiting, abdominal pain, altered mental status). Laboratory findings show hyponatremia, hyperkalemia, hypoglycemia, and elevated ACTH with low cortisol levels. While classically associated with meningococcal sepsis, it can also occur with other bacterial infections (Pseudomonas, Staphylococcus, Streptococcus pneumoniae), viral infections, or anticoagulant therapy. Diagnosis requires high clinical suspicion and is confirmed by imaging (CT/MRI showing bilateral adrenal hemorrhage) and hormonal studies. Treatment involves immediate IV corticosteroids (hydrocortisone), aggressive fluid resuscitation, vasopressors for shock, and appropriate antimicrobial therapy. Without prompt treatment, mortality approaches 100%.