Correspondence

Waterhouse–Friderichsen Syndrome

N Engl J Med 2001; 345:841September 13, 2001

Article

To the Editor:

The Waterhouse–Friderichsen syndrome was described by the British physician R.A. Waterhouse in 1911 as “a case of suprarenal apoplexy”1 and by C. Friderichsen, a Danish pediatrician, in 1918 as “Nebennierenapoplexie bei kleinen Kindern.”2 Waterhouse's description of an unvaccinated eight-month-old boy with a purpuric rash who died within four hours after admission included postmortem findings of bilateral adrenal hemorrhage. No causative organism was found, and the assumption that the disease was related to the unvaccinated state of the infant may have been erroneous. Nevertheless, the description of the syndrome has evolved to include fulminant purpuric meningococcemia associated with bilateral adrenal hemorrhage.

The Image in Clinical Medicine by Schoeller and Schmutzhard (May 3 issue)3 is of a 15-year-old boy with fulminant meningococcemia. No mention is made of the state of his adrenal glands. Therefore, his illness may not represent the complete syndrome that Waterhouse and Friderichsen independently described.

Paul Harris, M.D.
Angela Bennett, M.D.
SUNY Downstate Medical Center, Brooklyn, NY 11203-2098
pharris@downstate.edu

3 References

1. 1

   Waterhouse R. A case of suprarenal apoplexy. Lancet 1911;1:577-578
   CrossRef | Web of Science

2. 2

   Friderichsen C. Nebennierenapoplexie bei kleinen Kindern. Jahrb Kinderh 1918;87:109-125
   

3. 3

   Schoeller T, Schmutzhard E. Waterhouse-Friderichsen syndrome. N Engl J Med 2001;344:1372-1372
   Full Text | Web of Science | Medline

Author/Editor Response

Dr. Schoeller and colleagues reply:

To the Editor: Harris and Bennett note that in the original descriptions by Waterhouse and Friderichsen apoplexy of the adrenal glands was found and conclude that the symptom of adrenal hemorrhage is obligatory in the Waterhouse–Friderichsen syndrome. In our patient, a computed tomographic (CT) scan showed bilateral adrenal hemorrhage but no clinical or functional signs of adrenal insufficiency. Nevertheless, we initially administered a corticosteroid (dexamethasone), as is generally advised1 and as was advocated by Friderichsen in his later publications.2 There is a difference between adrenal apoplexy, the term used by the initial describers,3,4 and adrenal hemorrhage: the latter is not necessarily associated with functional adrenal insufficiency. Since adrenal hemorrhage is not thought to be specifically associated with the Waterhouse–Friderichsen syndrome5 but is related more to endotoxin, stress, anticoagulant therapy, or hypotension, we did not specifically refer to this CT finding.

Thomas Schoeller, M.D.
Angela Otto-Schoeller, M.D.
Gottfried Wechselberger, M.D.
University Hospital, A-6020 Innsbruck, Austria
thomas.schoeller@uibk.ac.at

5 References

1. 1

   Nelson J, Goldstein N. Nature of Waterhouse-Friderichsen syndrome: report of case with successful treatment with cortisone. JAMA 1951;146:1193-1197
   Web of Science | Medline

2. 2

   Friderichsen C. Waterhouse-Friderichsen syndrome. Acta Endocronologica 1955;18:482-492
   

3. 3

   Waterhouse R. A case of suprarenal apoplexy. Lancet 1911;1:577-578
   CrossRef | Web of Science

4. 4

   Friderichsen C. Nebennierenapoplexie bei kleinen Kindern. Jahrb Kinderh 1918;87:109-125
   

5. 5

   Xarli VP, Steele AA, Davis PJ, Buescher ES, Rios CN, Garcia-Bunuel R. Adrenal hemorrhage in the adult. Medicine (Baltimore) 1978;57:211-221
   Web of Science | Medline

Citing Articles (1)

Citing Articles

1. 1

   Cabot, Richard C.Harris, Nancy Lee, McNeely, William F., Shepard, Jo-Anne O., Ebeling, Sally H.Ellender, Stacey M.Peters, Christine C., Warren, H. Shaw Jr., Gonzalez, R. Gilberto, Tian, Di, . (2003) Case 38-2003. New England Journal of Medicine 349:24, 2341-2349
   Full Text