Vasculitides: F. Kawasaki’s Disease

Myones, Barry L.

Kawasaki’s disease (KD), once known as mucocutaneous lymph node syndrome, is a systemic inflammatory disorder occurring in children that is accompanied by vasculitis and a risk of coronary artery aneurysms. Other typical features of KD include spiking fevers, cervical lymphadenopathy, conjunctivitis, erythematous changes on the lips and in the oral cavity, dryness and cracking of the lips, a strawberry appearance to the tongue, and a polymorphous rash. Eighty percent of KD cases occur in children less than 5 years of age. Attempts to link KD definitively to some types of infection, particularly ones associated with superantigens, have thus far been unsuccessful. High dose aspirin and intravenous immune globulin (IVIG) are the cornerstones of therapy in KD. IVIG is essential to the prevention of coronary aneurysms. ■Years after KD has occurred during childhood years, some cases of myocardial infarction caused by thrombosis of coronary aneurysms have been reported.