Kawashaki disease (KD,mucocutaneous lymph node syndrome) is a generalized vasculitis of unknown cause Clinically It is characterized by fever(>5 days),polymorphous rashes,swelling of hands & feet, conjunctivitis,cracked lips, strawbery tongue, lymphadenopathy, epidermal digital peeling of fingertipe. Therapeutically,IVIG is recommended for reducing the risk of coronary artery dilation and aneurysm formation.Harada score could be used not only to predict coronary arterial lesion in KD,but alsodecide whether IVIG therapy should be administrated in early stage of KD.四川大学华西第二医院小儿科肖侠明

Atypical and incomplete Kawasaki disease.

Cimaz R, Sundel R. Best Pract Res Clin Rheumatol.2009 ;23(5):689-97.

Kawasaki disease (KD) is the most common systemic vasculitis in childhood after Henoch-Schonlein purpura, and the most common cause of acquired heart disease among children living in Western countries. Its diagnosis relies on clinical findings; laboratory tests are useful to rule out other causes of unexplained fever but are not specific for the diagnosis of KD. Numerous efforts to produce a diagnostic algorithm have been made, but without success. Expert opinion is therefore required in doubtful cases, especially those that lack classical criteria (the so-called atypical or incomplete cases). Renal, gastrointestinal, neurologic, pulmonary and ocular involvements have all been described. Infants may be at higher risk of complications since recognizing manifestations of the disease might be more difficult in this group. Approaches to treatment and follow-up of KD are changing in parallel with changes in concepts of what constitutes classical and incomplete KD. Guiding this evolution is the probability that the diagnosis is actually KD, the duration of the child's illness and the desired effects of therapy. Until a gold standard for diagnosing KD is available, these therapeutic decisions will continue to be made on an individual basis.

Kawasaki disease (KD) is a febrile systemic vasculitis complicated by coronary and peripheral arterial aneurysms in 20-35% of untreated patients. It is reported as the commonest cause of acquired heart disease in children in developed countries, and may be a risk for adult ischemic heart disease. Although KD has been reported all over the world, it is overexpressed among Asian populations, especially Japanese. The disease pathogenesis is still unknown and several theories have been proposed, including the possibility of an infection by a toxin-secreting microorganism and of a superantigen-driven process. Despite numerous efforts there is still no diagnostic test available for KD, and the diagnosis is based on clinical criteria after the exclusion of other diseases presenting with high persistent fever. Prompt diagnosis is critical, since the early administration of intravenous immunoglobulins and aspirin reduces the rate of coronary abnormalities to less than 5% of patients.