TABLE 1.

Clinical and Laboratory Features of Kawasaki Disease

Epidemiological case definition (classic clinical criteria)*
    Fever persisting at least 5 days
    Presence of at least 4 principal features:
        Changes in extremities
            Acute: Erythema of palms, soles; edema of hands, feet
            Subacute: Periungual peeling of fingers, toes in weeks 2 and 3
        Polymorphous exanthem
        Bilateral bulbar conjunctival injection without exudate
        Changes in lips and oral cavity: Erythema, lips cracking, strawberry tongue, diffuse injection of oral and pharyngeal mucosae
        Cervical lymphadenopathy (>1.5-cm diameter), usually unilateral
    Exclusion of other diseases with similar findings
Other clinical and laboratory findings
    Cardiovascular findings
        Congestive heart failure, myocarditis, pericarditis, valvular regurgitation
        Coronary artery abnormalities
        Aneurysms of medium-size noncoronary arteries
        Raynaud's phenomenon
        Peripheral gangrene
    Musculoskeletal system
        Arthritis, arthralgia
    Gastrointestinal tract
        Diarrhea, vomiting, abdominal pain
        Hepatic dysfunction
        Hydrops of gallbladder
    Central nervous system
        Extreme irritability
        Aseptic meningitis
        Sensorineural hearing loss
    Genitourinary system
        Urethritis/meatitis
    Other findings
        Erythema, induration at BCG inoculation site
        Anterior uveitis (mild)
        Desquamating rash in groin
Laboratory findings in acute Kawasaki disease
    Leukocytosis with neutrophilia and immature forms
    Elevated ESR
    Elevated CRP
    Anemia
    Abnormal plasma lipids
    Hypoalbuminemia
    Hyponatremia
    Thrombocytosis after week 1§
    Sterile pyuria
    Elevated serum transaminases
    Elevated serum gamma glutamyl transpeptidase
    Pleocytosis of cerebrospinal fluid
    Leukocytosis in synovial fluid
  • Patients with fever at least 5 days and <4 principal criteria can be diagnosed with Kawasaki disease when coronary artery abnormalities are detected by 2DE or angiography.

  • In the presence of ≥4 principal criteria, Kawasaki disease diagnosis can be made on day 4 of illness. Experienced clinicians who have treated many Kawasaki disease patients may establish diagnosis before day 4.

  • See Table 2.

  • § Some infants present with thrombocytopenia and disseminated intravascular coagulation.