In 1967, Kawasaki reported an acute, febrile, mucocutaneous condition accompanied by swelling of cervical lymph nodes that afflicted infants and young children in Japan. He called the entity mucocutaneous lymph node syndrome. In 1974, the condition was reported in Hawaii, and several cases have been reported subsequently from the continental United States and other countries. One percent to two % of patients had died suddenly of cardiac failure. Rickettsia-like bodies have been demonstrated from skin and lymph node biopsy specimens, and the pathologic features suggest a relationship to infantile periarteritis nodosa.