Kawasaki disease

Rarest of the rare disease..The `Kawasaki disease` (mucocutaneous lymph node syndrome) is a form of vasculitis identified by an acute febrile illness with multiple systems affected. The cause is unknown, but autoimmunity, infection, and genetic predisposition are believed to be involved. It affects mostly children between ages 3 months and 8 years; 80% are younger than age 5. It occurs more commonly in Japanese children or those of Japanese decent. It has seasonal epidemics, usually in late winter and early spring. It was first described in 1967 by Dr. Tomisaku Kawasaki in Japan.Although Kawasaki disease is a multisystem disease, the cardiovascular system appears to be the primary site with coronary artery vasculitis, aneurysm development, thrombosis, and myocardial thrombosis progressing over days to weeks. Kawasaki disease is diagnosed off the presence of symptoms rather than the results of tests. Symptoms include: conjunctivitis, rash, adenopathy, strawberry tongue, and rash on palms and soles of hands and feet. Four of these symptoms must be present, in addition to a fever which has lasted more than 5 days to confirm a diagnosis. Learn about atypical Kawasaki disease, and how health professional treat the disease using intravenous immunoglobulins (IVIG) and acetylsalicylic acid.Approximately 15% to 25% of patients develop cardiac complications (coronary thrombosis or rupture, myocardial infarction, heart failure, vasculitis of the aorta or peripheral arteries); however mortality is low.