Kawasaki disease was described in 1967. Although seen worldwide, its incidence is highest in Japan. It is encountered between 6 months to 5 years of age. Its etiology remains to be elucidated, but there is enough evidence that an unknown etiologic agent plays a role in the pathogenesis of the disease. Kawasaki disease is an inflammatory disorder, in which middle-sized arteries, like coronary vessels are involved. Its histopathology is identical to that of pediatric periarteritis nodosa. In its classic form there is fever, conjunctivitis, oral mucosal changes, findings concerning hands and feet, rash and lymphadenopathy. Its course consists of acute, subacute and convalescent phases. The diagnosis of classic Kawasaki disease necessitates the presence of fever of at least five day duration and four of the following criteria: conjunctivitis, oral mucosal changes, findings concerning hands and feet, rash and lymphadenopathy. Acute phase reactants and echocardiography are useful in diagnosis. Incomplete (atypical) Kawasaki disease may be confused with other diseases. If the patient characteristics do not fit well in another clinical entity, echocardiography is indicated and drug therapy is initiated. Therapy consists of aspirin plus intravenous immune globulin. In refractory patients immune globulin dose may be repeated. Coronary aneurysms may arise in 25% of children if left untreated. In half of these patients, the vessel diameter returns to normal within one year, but myocardial infarction due to obstruction is a worrisome possibility. Prevention will not be possible as long as the etiology remains obscure. (J Pediatr Inf 2007; 1: 20-6)
