A 26-year-old previously healthy man develops fever, purpuric rash, and hypotension progressing to shock over hours. Blood cultures grow Neisseria meningitidis. He dies despite antibiotics. At autopsy, both adrenal glands are replaced by massive hemorrhage. What is the name of this clinicopathologic syndrome?
- A Waterhouse-Friderichsen syndrome ✓
- B Addison disease
- C Conn syndrome
- D Sheehan syndrome
Explanation
Waterhouse-Friderichsen syndrome is overwhelming septicemia, classically meningococcal, producing bilateral adrenal hemorrhage and acute adrenal insufficiency with refractory shock and DIC related purpura. It is an acute catastrophic event, unlike chronic Addison disease, which results from gradual autoimmune destruction of the cortex. Conn syndrome is aldosterone producing adenoma causing hypertension, and Sheehan syndrome is postpartum ischemic necrosis of the anterior pituitary, so none of those involve acute bilateral adrenal hemorrhage.
Reference: Robbins and Cotran Pathologic Basis of Disease, 10th ed.
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