Adrenal haemorrhage / Waterhouse-Friderichsen Syndrome - Erler Zimmer

Adrenal haemorrhage / Waterhouse-Friderichsen Syndrome - Erler Zimmer

Clinical History

A 77-year-old man reported a 3-day history of abdominal and flank pain accompanied by fevers and rigors. He was 2 weeks post-operative from duodenal ulcer repair surgery. On examination, he displayed hypotension, hyperkalemia, hyponatremia, and a purpuric rash. Blood cultures revealed Escherichia coli. Despite treatment attempts, he failed to respond and succumbed to septic shock shortly after admission.

Pathology

The kidney and adrenal gland have been combined and mounted to display the cut surfaces. Extensive hemorrhage is evident in the adrenal medulla, with some blood extravasation into the periadrenal fat. This case illustrates adrenal hemorrhage in the context of severe septic shock, also known as 'Waterhouse-Friderichsen' syndrome.

Further Information

Waterhouse-Friderichsen syndrome is characterized by adrenal hemorrhage caused by overwhelming sepsis, leading to hypotensive shock, disseminated intravascular coagulation (DIC), and adrenocortical insufficiency. While more common in children, it rarely occurs in adults. Neisseria meningitidis is responsible for over 80% of adrenal hemorrhage cases, with other potential culprits including Streptococcus pneumoniae, Pseudomonas aeruginosa, Escherichia coli, Klebsiella pneumoniae, Haemophilus influenzae, and staphylococci.
The exact cause of the hemorrhage remains unclear and may be attributed to bacterial seeding of adrenal vessels, DIC, or endothelial dysfunction from inflammatory mediators or bacterial toxins. Bilateral adrenal hemorrhages can occur, originating in the medulla and extending outward to the cortex, potentially reaching the periadrenal fat. This progression leads to adrenal gland failure. Patients present with rapidly progressive septic shock, diffuse purpuric skin rash, and adrenal insufficiency crisis. Treatment involves supportive therapy, intravenous antibiotics targeting cultured organisms, and steroid therapy. The mortality rate exceeds 50%.