Kawasaki syndrome (the mucocutaneous lymph node syndrome)

Kawasaki syndrome is a newly-recognized clinical entity characterized by multisystem involvement. It has an acute onset and triphasic clinical course. Although essentially a self-limiting disease, permanent vascular damage, especially involving the coronary arteries, may result. Pathologically, the disease is characterized by widespread vasculitis. There is a monomodal age distribution, with peak occurrence during the first two years of life and few affected over the age of 8 years. Males outnumber females 1.5:1; persons of Japanese extraction are overrepresented compared with other races, caucasians underrepresented. Community-wide epidemics have been reported from diverse locations but there is no evidence for direct person-to-person transmission. Etiology remains unknown. Therapy is supportive and should be directed at careful clinical evaluation for cardiovascular abnormalities and antiplatelet aggregation therapy.