An 11-year-old boy presented to the emergency department with a 12-hour history of fever, rash over his trunk, vomiting, and diarrhea. In the emergency department, he had a fever to 39.3ºC., pulse 140/minute, respiratory rate 40/minute, and blood pressure 90/60 mm Hg. He had conjunctival injection and inflamed oral mucus membranes, and intense erythroderma (red skin) on his trunk and back (Figure 185-1). Laboratory tests revealed thrombocytopenia, transaminitis, and an elevated creatinine level that was twice normal for his age. He was given fluid resuscitation and was admitted to the pediatric intensive care unit, where he required several fluid boluses and inotropic support to maintain adequate blood pressure and perfusion. He was treated with vancomycin and clindamycin. Staphylococcus aureus was isolated from an infected wound on his lower extremity that he sustained from a sports injury a few days prior to his presentation.

Toxic shock syndrome (TSS) is an acute illness characterized by fever, rash, hypotension, and multi-organ system involvement that can progress to shock, renal failure, myocardial dysfunction, and adult respiratory distress syndrome. TSS was originally described in 1978 in children who had infection caused by Staphylococcus aureus, and has been well described in menstruating females using tampons.^1,2 A similar toxic shock-like syndrome has been described in association with group A streptococcal (GAS) infection.^3,4

Staphylococcal Toxic Shock

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  There is a strong correlation between menstruation, use of high absorbency tampons, and the development of TSS.⁵

  
  
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  Sixty percent of TSS cases occur in menstruating women while 40 percent occur in males and nonmenstruating females.⁶

  
  
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  More likely to occur in younger individuals who have not had previous exposure to TSS toxins and lack neutralizing antibody.⁷

  
  
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  Young women who have vaginal colonization with toxin-producing strains of S aureus and who have no antibody to TSS toxin 1 (TSST-1) are at highest risk of developing TSS during menstruation, especially with tampon use.

  
  
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  Children who have focal infection caused by S aureus can develop TSS. These infections may be clinically apparent, such as wound infections, skin abscesses, cellulitis, tracheitis, or may be occult, such as with sinusitis.

Streptococcal Toxic Shock-Like Syndrome

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  Usually related to GAS bacteremia, and in many cases often associated with cellulitis and necrotizing fasciitis.^3,4

  
  
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  Associated with varicella infections in which GAS secondarily infects skin lesions.

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  TSS is caused by S aureus strains that produce TSS toxin 1 or possibly other Staphylococcal enterotoxins.⁸

  
  
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  TSS toxins are thought to act as superantigens that stimulate the production of inflammatory mediators such as tumor necrosis factor and interleukin 1. These mediators cause capillary leakage that lead to hypotension and multisystem organ dysfunction.^9–11

  
  
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  The degree of hypotension predicts the extent of multisystem organ dysfunction.

  
  
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  Current clones of community-associated strains of methicillin-resistant S aureus (MRSA) rarely produce TSS toxin.¹²

  
  
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  Most cases of GAS toxic shock-like syndrome are due to strains of M types 1 and 3.

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  TSS is most common in young menstruating women using high absorbency tampons.

  
  
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  The lack of antibody to TSS-toxin 1 is a risk factor for infection.

  
  
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  Varicella infection is an important risk factor for Streptococcal toxic shock-like syndrome (Figure 185-2).

Clinical Features

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  Formal case definitions for TSS and Streptococcal toxic shock-like syndrome have been published (Tables 185-1 and 185-2).

  
  
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  Acute onset of fever, sore throat and myalgia are the first symptoms.

  
  
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  Gastrointestinal manifestations, especially profuse diarrhea, are common.

  
  
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  Rash is erythrodermal or scarlatiniform in nature (Figures 185-1 and 185-3).

  
  
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  Symptoms of hypotension are often present (dizziness or fainting).

  
  
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  “Strawberry tongue” (Figure 185-4), nonpurulent conjunctival injection (Figure 185-5), and pharyngeal inflammation are common features on physical examination.

  
  
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  Altered consciousness is a sign of hypotension.

  
  
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  Desquamation of the skin occurs 7 to 21 days after the onset of illness; this commonly occurs in a full-thickness, sheet like manner (Figure 185-6).

  
  
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  The clinical features of GAS toxic shock-like syndrome are similar to that of Staphylococcal toxic shock; however, GAS toxic shock is more often preceded by wound infection, cellulitis or pneumonia (Figure 185-7).