Table 1. Diagnostic guidelines for Kawasaki disease from the scientific statement of the American Heart Association

Principal clinical features• Erythema and cracking of lips, strawberry tongue, and/or erythema of oral and pharyngeal mucosa• Bilateral bulbar conjunctival injection without exudate• Rash: maculopapular, diffuse erythroderma, or erythema multiforme-like• Erythema and edema of the hands and feet in acute phase and/or periungual desquamation in the subacute phase• Cervical lymphadenopathy (≥ 1.5 cm diameter), usually unilateral
Classic Kawasaki disease1. Classic KD is diagnosed in the presence of fever for at least 5 d (the day of fever onset = the first day of fever) together with at least 4 of the principal clinical features.2. In the presence of ≥ 4 principal clinical features, particularly when redness and swelling of the hands and feet are present, the diagnosis of KD can be made with 4 d of fever.
Incomplete Kawasaki diseaseIncomplete KD is suspected in1. Children with fever ≥ 5 days and 2 or 3 principal clinical features or2. Infants with fever for ≥ 7 days without other explanation A. If CRP ≥ 3 mg/dL and/or ESR ≥ 40 mm/hr : 3 or more laboratory findings1) OR positive echocardiogram2) = Incomplete KD B. If CRP< 3 mg/dL and ESR < 40 mm/hr : Serial clinical and laboratory re-evaluation if fevers persist. Echocardiogram if typical peeling develops. : If ≥ 4 principle features, ≥ 3 laboratory findings OR positive echocardiogramb = Incomplete KD
Anemia for age, platelet count of ≥ 450,000 after the 7th day of fever, albumin ≤ 3 g/dL, elevated ALT level, WBC count of ≥15,000/mm3, urine ≥ 10 WBC/HPF.
Echocardiography is considered positive for purposes of this algorithm if any of 3 conditions are met: Z score of left anterior descending coronary artery or right coronary artery ≥ 2.5; coronary artery aneurysm is observed; or ≥ 3 other suggestive features exist, including decreased left ventricular function, mitral regurgitation, pericardial effusion, or Z scores in left anterior descending coronary artery or right coronary artery of 2 to 2.5.
CRP: C-reactive protein; ESR: erythrocyte sedimentation rate; ALT: Alanine Aminotransferase; HPF: high power field.