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Clinical Features, Pathogenesis, and Recent Microbiological and Biochemical Developments

Peter M. Elias, MD; Peter Fritsch, MD; Ervin H. Epstein, Jr, MD

Arch Dermatol. 1977;113(2):207-219.

Abstract
• The essential clinical features of staphylococcal scalded skin syndrome (SSSS) and other forms of toxic epidermal necrolysis (TEN) are contrasted. Whereas TEN is a devastating disease of multiple causes and of high fatality affecting all age groups, SSSS comprises many clinical entities that occur primarily in early childhood and is caused by certain phage group 2 staphylococci. Because of the high cleavage plane, the barrier is only transiently perturbed, and rapid recovery is the rule. Although the early stages of SSSS may resemble other widespread dermatoses clinically, the correct diagnosis is suggested, even prior to frank exfoliation, by the presence of cutaneous tenderness and a positive Nikolski sign. However, rapid bedside confirmation is now possible with exfoliative cytology and frozen sections. Recent availability of in vivo and in vitro animal models of SSSS have advanced the knowledge of the disease: the responsible epidermolytic toxin has been characterized, and the purely extracellular pathogenesis of SSSS has been established. The epidermolytic toxin is strikingly species and tissue specific, attacking only certain keratinizing epithelia of mice, hamsters, monkeys, and man. The lower incidence of SSSS in adults is primarily due to a superior capacity to metabolize and excrete the toxin, as well as more efficient immune capabilities. The mechanisms of epidermolytic toxin action and the molecular site of action are still the focus of active investigation.

(Arch Dermatol 113:207-219, 1977)

Author Affiliations
From the Veterans Administration Hospital (Dr Elias) and the Departments of Dermatology, University of California School of Medicine (Dr Epstein), San Francisco, and the I. Hautklinik, University of Vienna (Dr Fritsch).

Footnotes
Accepted for publication July 23, 1976.

Reprint requests to Dermatology Section, Veterans Administration Hospital, 4150 Clement St, San Francisco, CA 94121 (Dr Elias).

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