[Atypical Kawasaki: the often missed diagnosis]

ABSTRACT

Kawasaki disease is an acute febrile vasculitis of childhood first described by Dr. Tomisaku Kawasaki in Japan in 1967. The disorder occurs worldwide, with Asians at highest risk. Approximately 20% of untreated patients develop coronary artery abnormalities including aneurysms, with the potential for the development of coronary artery thrombosis or stenosis, myocardial infarction, aneurysm rupture, and sudden death. Kawasaki disease has replaced acute rheumatic fever as the leading cause of acquired heart disease in children. The disease is characterized by fever, bilateral nonexudative conjunctivitis, erythema of the lips and oral mucosa, changes in the extremities, rash, and cervical lymphadenopathy. The classic diagnosis of Kawasaki disease has been based on the presence of fever and >/= 4 of the 5 principle clinical features. During the past few years there have been several reports of patients with coronary aneurysms corresponding to changes in Kawasaki disease who did not exhibit the other required symptoms. The diagnosis of Atypical Kawasaki, in addition to coronary aneurysms, requires the presence of three to four of the major symptoms. Atypical Kawasaki is more common in infants than in older children, making accurate diagnosis and timely treatment especially important in these young patients who are at substantial risk of developing coronary abnormalities. Therefore, Kawasaki disease should be considered in an infant with prolonged, high fever in spite of atypical or incomplete presentation and echocardiography may help us to begin earlier treatment in these high risk patients