Kawasaki Disease
This 5-year-old child has Kawasaki disease until proven otherwise, and requires immediate echocardiography and treatment with intravenous immunoglobulin (IVIG) 2 g/kg plus high-dose aspirin (80-100 mg/kg/day divided into four doses) to prevent coronary artery aneurysms. 1
Clinical Presentation Matches Classic Kawasaki Disease
The constellation of findings in this child is highly characteristic:
- Fever for ≥5 days is the cornerstone diagnostic feature 1
- Rash on palms and soles represents the extremity changes (erythema and edema of hands/feet with sharp demarcation at wrists/ankles) that is one of the five principal clinical features 1, 2
- Elevated WBC count (leukocytosis with neutrophil predominance) is typical, with approximately 50% of patients having counts >15,000/mm³ 1
- Elevated ESR is nearly universal in Kawasaki disease, often >40 mm/hour and commonly ≥100 mm/hour 1
- Thrombocytosis (high platelets) typically appears in the second week after fever onset, which fits this presentation 1
Diagnostic Criteria
The diagnosis requires fever ≥5 days plus ≥4 of the following 5 principal features: 1
- Bilateral bulbar conjunctival injection without exudate
- Oral mucosal changes (erythema/cracking of lips, strawberry tongue, pharyngeal erythema)
- Polymorphous rash (maculopapular, diffuse erythroderma, or erythema multiforme-like)
- Extremity changes (erythema and edema of hands/feet in acute phase; periungual desquamation in subacute phase)
- Cervical lymphadenopathy (≥1.5 cm diameter)
The child already has confirmed fever, extremity changes (palms/soles rash), and laboratory evidence of systemic inflammation. 1
Critical Laboratory Findings Supporting the Diagnosis
The laboratory profile is classic for Kawasaki disease: 1
- Leukocytosis with immature and mature granulocyte predominance
- Elevated acute phase reactants (ESR and CRP are nearly universal)
- Thrombocytosis appearing after day 7 of illness (>450,000/mm³ is a supplemental criterion)
- Additional supportive findings may include: anemia for age, hypoalbuminemia (<3.0 g/dL), elevated ALT, and sterile pyuria (>10 WBC/hpf) 1
Why This Matters: Coronary Artery Complications
Without treatment, 15-25% of children develop coronary artery aneurysms or ectasia, which can lead to myocardial infarction, sudden death, or ischemic heart disease. 1, 3, 4 Treatment with IVIG within 10 days of fever onset reduces this risk from 25% to approximately 5%. 2
Immediate Management Algorithm
Step 1: Obtain echocardiography immediately 1
- Even though aneurysms rarely form before day 10, early findings may include perivascular brightness, ectasia, lack of tapering, decreased LV function, mitral regurgitation, or pericardial effusion 1
- If coronary abnormalities are present with only 3 clinical features, the diagnosis is confirmed 1
Step 2: Initiate treatment without delay 1
- IVIG 2 g/kg as a single infusion (first-line, Level A evidence)
- High-dose aspirin 80-100 mg/kg/day divided into four doses given concurrently with IVIG
- Treatment should begin as soon as the diagnosis is suspected, ideally within 10 days of fever onset 1, 2
Common Diagnostic Pitfalls to Avoid
Do not dismiss this as a viral illness or drug reaction simply because the full criteria aren't initially apparent. 1 Critical mistakes include:
- Misdiagnosing as bacterial lymphadenitis when cervical adenopathy is prominent, then attributing subsequent rash to antibiotic reaction 1
- Mistaking sterile pyuria for partially treated UTI 1
- Missing the diagnosis in infants <6 months who often present with incomplete Kawasaki disease and have the highest risk for coronary complications 1, 5, 4
- Waiting for all five clinical features to appear simultaneously—they may evolve over days, requiring careful history-taking 1
Differential Diagnosis Considerations
Features that would argue against Kawasaki disease include: 1
- Exudative conjunctivitis or pharyngitis
- Discrete intraoral ulcerations
- Vesicular or bullous rash
- Generalized (rather than cervical) lymphadenopathy
The absence of vesicular lesions distinguishes this from hand-foot-mouth disease, which presents with discrete vesicles rather than diffuse erythema. 6 The duration of fever (≥5 days) and laboratory findings (markedly elevated ESR/platelets) distinguish this from viral exanthems like roseola. 7
Risk Stratification
This child is at particularly high risk if: 1
- Fever persists beyond 10 days (powerful predictor of coronary aneurysms)
- Age <1 year (paradoxically higher aneurysm rate despite incomplete presentations)
- Male sex
- Specific laboratory thresholds: WBC >12,000/mm³, platelets after day 7 >450,000/mm³, hemoglobin <10 g/dL, albumin <3.5 g/dL
All patients diagnosed with Kawasaki disease should receive IVIG regardless of risk stratification, as scoring systems have imperfect predictive value. 1