-vasculitis; inflammation of vessel walls

-febrile illness 

-self-limiting

-necrotizing vasculitis 

-M:F = 1.36:1 (Japan)

-peak age at onset <5 (Japan: <1 year, US:2-3 years)

-incidence: 15-150/100,000 toddlers/year

-fever (> or = 39-40 degrees C) @ least 5 days

AND

> or = to 4 major clinical features:

-bilateral conjunctival infection

-oropharyngeal mucosa changes

-cervical lymphadenopathy

-polymorphous rash

-changes in peripheral extremities 

-viral illnesses such as Measles, EBV, Adenovirus

-Toxin mediated such as Post strep scarlet fever, TSS

-Drug reactions: Steven Johnson syndrome

-Autoimmune: systemic JRA, PAN

CBC: white cell and platelets are high

-elevated ESR

-elevated C-reactive protein 

-chemistries are normal

-urine may be normal or show sterile pyuria 

-**need to do cultures/serologies to R/O infection

-IV immunoglobins

-NSAIDS

-high dose aspirin until fever resolves

-low dose until ESR normalizes 

-most serious: cardiac disease

-leading cause of acquired heart disease in children

- complications include:

-myocarditis, pericarditis, valvular heart disease (usually mitral or aortic regurgitation), and coronary arteritis

-abrupt onset, often post URI or GI illness

-high persistent fever; irritability

-in 3-4 days, occular, mucocutaneous changes, lymphadenitis, and erythema/edema of hands and feet

-potential for carditis

-abdominal pain, hydrops gall bladder