Thirty-six years ago, Tomisaku Kawasaki penned the first description of a quixotic illness that he called mucocutaneous lymph node syndrome. Today this illness has been described in almost every country of the world. In studies from North America, Japan, and Western Europe, Kawasaki syndrome has replaced rheumatic fever as the most common cause of acquired heart disease. Despite the importance of Kawasaki syndrome as a cause of disease in children, a surprising number of aspects of this syndrome remain ill-defined. Because the etiology remains incompletely understood, the diagnosis is based on history and physical examination. Because the symptoms of Kawasaki syndrome are not unique and diagnosis can be difficult, it is likely that many cases of incomplete or atypical Kawasaki syndrome, as well as some presentations of classic Kawasaki syndrome, remain undiagnosed and untreated, and the child remains at risk of undetected coronary artery disease. Therapy with intravenous...

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