Case report of an unusual presentation of Staphylococcus aureus induced toxic shock syndrome/hyperimmunoglobulinemia E syndrome

Harry S. Jacob, Gregory M. Vercellotti, Donald Y.M. Leung, Patrick M. Schlievert

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Rationale:Toxic shock syndrome (TSS) typically is an acute onset multi-organ infection caused by TSS toxin-1 producing Staphylococcus aureus. Herein we describe a highly unusual case report.Patient concerns:A male patient self-referred to the University of Minnesota Hospital with a chronic history of S aureus infection with accompanying fever, hypotension, and nonhealing, football-sized lesion on his leg.Diagnosis:An unusual case presentation of TSS/hyperimmunoglobulin E syndrome is described. The patient had a leg wound from which TSS toxin-1 S aureus was isolated. The patient exhibited characteristic skewing of T cells to those with variable region, β-chain T cell receptor-2. Other patients have been seen with related presentations.Interventions:The following therapeutic regimen was instituted: vigorous antibacterial scrubs several times daily plus intravenous Ancef 3 days each month; intravenous infusions of immunoglobulin G infusions (28 gm) every 3 weeks; and weekly subcutaneous injections of recombinant granulocyte colony-stimulating factor.Outcome:Improvement was obvious within 3 months: no further cellulitic episodes occurred; the patient regained 95 pounds in 9 months; blanching and cyanosis of fingers disappeared within 3 months as did intractable pain although mild hypesthesias continued for 2 years; erythroderma resolved, and repeat skin biopsies performed after 2 years no longer demonstrated T cell receptor skewing. Although IgE levels have not completely returned to normal, the patient remains in excellent health.Lessons:We propose that staphylococcal TSST-1 was responsible for the serious problems suffered by this patient as suggested by the following features: rapid onset of chronic, life-threatening, disorder that began with an episode of staphylococcal sepsis; the extraordinary elevation of IgE levels in this previously non-atopic individual; the acquired severe granulocyte chemotactic defect that accompanied this hyperimmunoglobulinemia ("Job Syndrome") with its accompanying wound-healing defect; and the striking diffuse erythroderma, including palmar erythema ("Red Man Syndrome") with hypotension and fever that also characterizes TSS.

Original languageEnglish (US)
Pages (from-to)E19746
JournalMedicine (United States)
Issue number15
StatePublished - Apr 28 2020

Bibliographical note

Funding Information:
This work was supported by research grants from the University of Minnesota and USPHS research grant AR41256 to DYM Leung.

Publisher Copyright:
Copyright © 2020 the Author(s). Published by Wolters Kluwer Health, Inc.


  • Staphylococcus aureus
  • hyperimmunoglobulin E syndrome
  • toxic shock syndrome


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