A 6-year-old boy with fulminant meningococcaemia develops sudden shock, widespread purpura and bilateral flank pain. CT shows bilateral enlarged adrenal glands with haemorrhage. Autopsy would demonstrate haemorrhagic destruction of the adrenal cortex. This presentation is classically known as:

• A Congenital adrenal hyperplasia with salt wasting
• B Addisonian crisis secondary to autoimmune adrenalitis
• C Waterhouse-Friderichsen syndrome ✓
• D Nelson syndrome

Explanation

Waterhouse-Friderichsen syndrome is overwhelming sepsis, classically meningococcal, producing bilateral adrenal haemorrhage with cortical destruction, acute adrenal insufficiency, shock and DIC manifesting as purpura. Autoimmune adrenalitis causes chronic primary adrenal insufficiency with atrophy rather than haemorrhage. Salt-wasting CAH presents in infancy with hyperkalaemia, and Nelson syndrome follows bilateral adrenalectomy for Cushing disease with an enlarging ACTH adenoma.

Reference: Robbins and Cotran Pathologic Basis of Disease, 10th ed.

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Written and medically reviewed by the StethoPrep medical team.