Kawasaki Disease: Diagnosis and Treatment
Primary Diagnosis
This 2-year-old partially vaccinated male with prolonged fever, diarrhea, dysuria, phimosis, lethargy, irritability, and diffuse rash most likely has incomplete Kawasaki disease, which requires immediate IVIG and high-dose aspirin to prevent coronary artery aneurysms. 1
Clinical Reasoning for Kawasaki Disease
The constellation of symptoms strongly suggests incomplete Kawasaki disease rather than infectious gastroenteritis:
- Prolonged fever with systemic symptoms (lethargy, irritability) in a young child is the hallmark presentation, particularly in children <2 years who frequently present with incomplete forms 1
- Dysuria with sterile pyuria is a common pitfall—this is often misattributed to urinary tract infection when it actually represents urethritis from Kawasaki disease 1
- Diffuse rash combined with fever represents one of the principal clinical criteria 1
- Gastrointestinal symptoms (diarrhea) occur in many Kawasaki patients and can mislead clinicians toward infectious diagnoses 1
- Phimosis likely represents genital inflammation, which is a recognized manifestation of Kawasaki disease 1
Immediate Diagnostic Workup
Laboratory Evaluation Required
- CRP and ESR to assess inflammatory markers—if CRP ≥3.0 mg/dL and/or ESR ≥40 mm/hr, proceed with additional testing 1
- Complete blood count looking for anemia, platelet count ≥450,000 after day 7 of fever, and WBC ≥15,000/mm³ 1
- Comprehensive metabolic panel checking for albumin <3.0 g/dL and elevated ALT 1
- Urinalysis to document sterile pyuria (≥10 WBC/hpf without bacterial growth) 1
- Echocardiogram immediately to assess for coronary artery abnormalities—Z scores ≥2.5 for LAD or RCA have very high specificity for diagnosis 1
Diagnostic Algorithm for Incomplete Kawasaki Disease
If the patient has fever ≥5 days with 2-3 compatible clinical criteria (in this case: rash, irritability/behavioral changes, possible mucosal changes):
- Check inflammatory markers first 1
- If CRP ≥3.0 mg/dL or ESR ≥40 mm/hr, count supplemental laboratory findings 1
- If ≥3 supplemental laboratory findings are positive OR echocardiogram shows coronary abnormalities, treat as Kawasaki disease 1
Treatment Protocol
Primary Treatment: IVIG and Aspirin
- Intravenous immunoglobulin (IVIG) 2 g/kg as a single infusion over 10-12 hours within 10 days of fever onset (ideally within 7 days) to reduce risk of coronary artery aneurysms from 25% to <5% 1
- High-dose aspirin 80-100 mg/kg/day divided every 6 hours until patient is afebrile for 48-72 hours, then reduce to low-dose aspirin 1
- Low-dose aspirin 3-5 mg/kg/day once daily continued for 6-8 weeks if no coronary abnormalities, or indefinitely if coronary aneurysms develop 1
Supportive Care During Evaluation
- Aggressive oral rehydration with reduced osmolarity ORS for the diarrhea component, replacing 10 mL/kg for each watery stool 1, 2
- Continue age-appropriate diet as tolerated to maintain nutritional status 3
- Monitor for dehydration which increases morbidity risk, especially in young children 1
Critical Pitfalls to Avoid
Common Diagnostic Errors
- Never dismiss prolonged fever in infants and young children as simple viral illness—infants <6 months are at highest risk for incomplete Kawasaki disease and coronary complications 1
- Never attribute dysuria and pyuria solely to UTI without considering Kawasaki disease in the context of prolonged fever and other systemic symptoms 1
- Never delay treatment waiting for "complete" criteria—incomplete Kawasaki disease has the same cardiovascular sequelae as complete disease 1
- Never misattribute rash and mucosal changes to antibiotic reaction when the patient has prolonged fever—this is a classic pitfall that delays Kawasaki diagnosis 1
Treatment Errors to Avoid
- Never withhold IVIG beyond day 10 of fever as this is the critical window for preventing coronary damage 1
- Never use antibiotics empirically for the diarrhea without confirmed bacterial pathogen, as this promotes resistance and provides no benefit for viral or Kawasaki-related GI symptoms 1, 2
- Never use loperamide or antimotility agents in a febrile child with systemic symptoms, as these are contraindicated when fever is present 3, 4
Alternative Diagnoses to Consider
If Kawasaki disease is ruled out by negative inflammatory markers and normal echocardiogram:
- Infectious gastroenteritis would require stool culture, multiplex PCR panel, and Shiga toxin testing given fever and diarrhea 1
- Urinary tract infection should be confirmed with urine culture, though sterile pyuria argues against this 5
- Viral exanthem with concurrent gastroenteritis is possible but would not explain the dysuria and systemic toxicity 1
However, the combination of prolonged fever, systemic symptoms, rash, and dysuria in a 2-year-old makes incomplete Kawasaki disease the most likely diagnosis requiring urgent treatment to prevent life-threatening coronary complications. 1