More than half a century has elapsed since Prof. Tomisaku Kawasaki’s did the first description of a unique disease. He saw the first 4-year-old patient with fever and rash in 1961…. At that time, he described the diagnosis “unknown”. The published paper title was Infantile acute febrile mucocutaneous lymph node syndrome with specific desquamation of the fingers and toes. Clinical observation of 50 cases. From This “unknown diagnosis”, which now we call Kawasaki disease (KD) until the current era, this vasculitis of unknown cause became the leading cause of acquired heart disease among children in United States.

Historically, the presence of coronary abnormalities was not noticed until patients died suddenly of cardiac complications. An angiographic study of 1100 patients showed coronary artery lesions in 24%, with aneurysms in 8% and a number of patients with stenoses and occlusions. As soon as the Coronary arteries became the key structure for risk stratification, treatment and outcome, extensive research and worldwide effort has been done targeting the correct diagnostic. Unfortunately, to make things more challenging, there are forms of “uncomplete KD” which overlaps with other forms of febrile Exanthematous illness in children.

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