Elsevier

Mayo Clinic Proceedings

Volume 76, Issue 11, November 2001, Pages 1167-1170
Mayo Clinic Proceedings

Case Report
Waterhouse-Friderichsen Syndrome After Infection With Group A Streptococcus

https://doi.org/10.4065/76.11.1167Get rights and content

We report a case of Waterhouse-Friderichsen syndrome associated with group A streptococcus (GAS) toxic shock syndrome in a previously healthy man. The patient presented with neck pain and fevers of 2 days’ duration. Computed tomography of the neck revealed a mass in the retropharyngeal space, suggesting an abscess. Despite prompt treatment with appropriate antibiotics, the patient experienced a fulminant course and died within 8 hours of presentation. Antemortem blood cultures grew GAS positive for exotoxins A, B, and C. Postmortem examination revealed bilateral adrenal hemorrhage, consistent with Waterhouse-Friderichsen syndrome. Immunohistochemical analysis of the adrenal glands revealed the presence of GAS antigens. However, no disseminated intravascular coagulation was evident. This case demonstrates that adrenal hemorrhage can occur without associated coagulopathy and may result directly from the action of bacterial toxins.

Section snippets

REPORT OF A CASE

A 32-year-old previously healthy man presented to the emergency department because of severe neck pain, odynophagia, and fevers of 2 days’ duration. He denied having any recent history of dental procedures or bacterial pharyngitis. His only ill contact was his sister, who had a sore throat. He specifically denied use of nonsteroidal anti-inflammatory drugs or other medications. He denied intravenous drug use and reported an allergy to penicillin.

Physical examination revealed a moderately

DISCUSSION

Our patient had a rapidly progressive course despite prompt administration of appropriate antibiotics. Antemortem blood cultures confirmed GAS bacteremia. In addition, autopsy findings revealed bilateral adrenal hemorrhage consistent with Waterhouse-Friderichsen syndrome. Based on a MEDLINE search of cases in the English language since 1966, this is the third reported case of Waterhouse-Friderichsen syndrome associated with GAS and the first case in an adult.4,5

The earliest reports of this

CONCLUSION

In this patient we documented the presence of GAS in the blood and the presence of GAS antigens in the tissues, as confirmed by immunohistochemistry studies. This case is unique in that our patient had no laboratory evidence of DIC and no dermatologic or pathologic findings consistent with DIC, suggesting that the adrenal hemorrhage was likely mediated by a bacterial toxin.

We thank Patrick M. Schlievert, PhD, University of Minnesota, Minneapolis, for toxigenicity testing of the patient isolate

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