Kawasaki syndrome is a generalized vasculitis involving small and medium size arteries, with characteristic involvement of the coronary arteries. There is growing evidence to suggest that Kawasaki syndrome is caused by superantigen bacterial toxins, which stimulate large populations of T cells expressing particular T-cell receptor b chain variable-gene segments. Superantigen stimulation induces massive proliferation and expansion of the target T cells, with subsequent production of proinflammatory cytokines. Vascular endothelial cells are recruited into this inflammatory process with resulting vascular damage. Toxins elaborated by Staph. aureus and Streptococcus pyogenes are known to possess superantigen properties. There is increasing evidence to suggest that these organisms elaborate superantigen toxins in children with Kawasaki syndrome.
Epidemiologically, Kawasaki syndrome affects 3000 to 5000 children annually in the United States. There is a male-to-female preponderance of 1.5 to 1. The peak age of onset is 1 to 2 years, with 80 percent of patients being less than 4 years of age.
The diagnosis of Kawasaki syndrome is established by the presence of clinical criteria listed in lab'eJ32-3. The fever is high, spiking, and prolonged, persisting for 1 to 2 weeks in untreated patients. The conjunctivitis is nonpurulent and bilateral and has an onset shortly after the appearance of the fever. The oropharyngeal features are prominent during the acute febrile period. The extremities, particularly the palms and soles, are often quite painful. The polymorphous rash is most commonly a raised, deep-red, plaquelike eruption. Less commonly, it may be scarlatiniform, a morbilliform maculopapular rash, or even a fine pustular eruption. It is most widespread on the trunk and proximal extremities, with particular involvement of the perineum. At least one cervical lymph node measuring 1.5 cm in diameter is necessary to fill the lymphadenopathy criterion. There are a wide variety of associated findings in Kawasaki syndrome, as illustrated in Iable.132-4.
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