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Patient Story
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An 18-month-old girl is admitted to the hospital with fever, irritability, and a tender skin rash on her face. She also has developed facial swelling bilaterally and perioral crusting (Figure 105-1). Over the next 24 hours, the rash spreads to her neck and trunk and she develops flaccid blisters on the areas of rash on her neck and trunk. When gentle friction is applied to involved areas of the skin, the skin easily sloughs superficially (Nikolsky sign). She is treated with intravenous anti-staphylococcal antibiotics and recovers completely. A culture taken from her nares grows Staphylococcal aureus.

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FIGURE 105-1

18-month-old girl with SSSS. Note the edema of the face, perioral crustiness, and extension of the erythema to the neck and trunk. Also note the thin desquamation on the chest and axilla. (Used with permission from Camille Sabella, MD.)

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Introduction
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Staphylococcal scalded skin syndrome (SSSS) is a toxin-mediated illness mediated by exfoliative toxins A and B of Staphylococcus aureus.

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Synonyms
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Ritter syndrome (SSSS in neonates and young infants).

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Epidemiology
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  • Occurs mainly in children under 5 years of age.

  • May also occur in neonates after becoming colonized or infected with a toxin-producing strain of S aureus (Ritter syndrome).

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Etiology and Pathophysiology
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  • Exfoliative toxins A and B of S aureus are responsible for the manifestations of the illness.1,2

  • Mostly caused by S aureus belonging to phage group II, types 71 and 55.

  • Toxins are hematogenously spread and produce the fever and characteristic rash.

  • Toxin targets desmoglein 1, resulting in cleavage of the epidermis in a superficial location.3,4

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Risk Factors
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  • Primary infection with toxin-producing S aureus at sites other than the skin, such as the umbilicus (in neonates), nasopharynx, or conjunctiva.

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Diagnosis
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Clinical Features
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  • Fever, irritability, and tender erythema of the skin are common.

  • Facial edema with perioral and periocular crustiness is typical and may be the primary clinical features (Figures 105-2 to 105-4).

  • Flaccid blisters and superficial erosions form within 12 to 24 hours, and which progress to become widespread superficial peeling of the skin (Figure 105-5).

  • Gentle sheering of the skin often results in widespread separation (Nikolsky sign).

  • Desquamation of the skin often follows at the involved skin sites (Figure 105-6).5,6

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FIGURE 105-2

One-month-old infant with SSSS. Note the crustiness on the face and facial edema. (Used with permission from Charles B. Foster, MD.)

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FIGURE 105-3

Infant with edema and crustiness on the ...

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