Case: 14mo F with Rash

By Schon Roberts MD PGY-3

14 mo F, seen 3 days ago in ED with fever and possible UTI p/w new onset rash. She was placed on Omnicef and followed up with her regular doctor today. He sent her for evaluation. Parents state that she has had swelling of her hands and feet. Mother states that she has had a fever for 5 days. Fever was TMax 103 F. She has had adequate PO intake with good urine output and stooling. Parents deny URI Symptoms, N/V/D, or recent travel. Vital Signs show a pulse of 179, RR of 28, Temperature of 102.1 F, and patient has a spO2 of 99% on RA. Physical exam is remarkable for injected sclera, swelling of her hands and the dorsum of her feet. She has an erythematous rash on her trunk and extremities that blanches. It spares the palms and soles. Lungs are CTABL and cardiac exam is unremarkable. What is the best treatment option for this child?
 
A. Broaden antibiotic coverage for meningitis to include listeria coverage, LP, and admit to the hospital
B. Discharge home; Pt has a URI
C. PO Tylenol, wait for defervescence, and D/C if improved
D. Obtain an echocardiogram, start on high dose ASA, IVIG, and admit
E. Start on steroids and admit with nephrology consult

  Answer
D. Obtain an echocardiogram, start on ASA and IVIG, and admit
 
 
Pathophysiology
 

• Kawasaki Disease is a rare vasculitis of unknown etiology.
• Many have postulated that this is in part due to autoimmune response after an infectious trigger.
• Incidence is around 67 in 100,000 in the United States per year. Endemic areas of the world include the orient in countries like Japan were incidence has been estimated at 124 per 100,000.
• Vasculitis results in a myriad of symptoms to include diarrhea, arthralgia, fever, myocarditis, pericarditis, and coronary artery aneurysm.

  
Symptoms/Exam Findings
 

• Altered Mental Status
• Conjunctival Injection
• LAD of the Neck
• Strawberry Tongue
• Ungual Desquamation
• Swollen Extremities
• Fever
• Uveitis
• Maculo-paplar Rash​

Labs

• Echocardiogram
• ESR/CRP
• WBC
• CMP
• UA

 
Diagnosis  ​

Incomplete Kawasaki Disease
 
Many patients present without all findings of Kawasaki Disease. To make the Dx:
 
Three or more abnormal lab finding
•       Elevated CRP/ESR

• WBC >15,000
• Normocytic, normochromic anemia for age
• Platelets >450,000
• Pyuria
• ALT >50
• Albumin <3

Abnormal Echo- Coronary Aneurysms
Fever for >7 days w/o plausible different Dx
 
Treatment

• 2g/kg IVIG x 1 dose
  • Studies show inverse relationship between IVIG and incidence of Coronary Artery Aneurysm.
• 80-120 mg/kg/day ASA divided into 4 doses
• Supportive Care

​High vs. low dose IVIG
Randomized multicenter Controlled Study showed:

• Decreased incidence of Coronary Artery Aneurysms with IVIG of 2 gm/kg when compared with 1, 1.2, and 1.6 gm/kg IVIG
• Decreased incidence of Coronary Artery Aneurysms with 80-120 mg/kg/day ASA when compared with 30-50.

Clinical Pearls

• Suspect Kawasaki Disease in any child with fever of >5 days
• Fever in addition to 4 of the above criteria make the diagnosis of Kawasaki Disease
• Most feared outcome in Kawasaki Disease is Coronary Artery Aneurysms
• Treatment should include 2 gm/kg IVIG and 80-120 mg/kg/day ASA
• Suspect Incomplete Kawasaki Disease in a patient who does not meet all criteria and treat the same

 
References
1."Newburger JW, Takahashi M, Gerber MA, et al. Diagnosis, treatment, and long-term management of Kawasaki disease: a statement for health professionals from the Committee on Rheumatic Fever, Endocarditis and Kawasaki Disease, Council on Cardiovascular Disease in the Young, American Heart Association. Circulation 2004; 110:2747. 
2. Furusho K, Kamiya T, Nakano H, et al. High-dose intravenous gammaglobulin for Kawasaki disease. Lancet 1984; 2:1055.
 3.Newburger JW, Takahashi M, Burns JC, et al. The treatment of Kawasaki syndrome with intravenous gamma globulin. N Engl J Med 1986; 315:341.
4. Newburger JW, Takahashi M, Beiser AS, et al. A single intravenous infusion of gamma globulin as compared with four infusions in the treatment of acute Kawasaki syndrome. N Engl J Med 1991; 324:1633.
 5. Durongpisitkul K, Gururaj VJ, Park JM, Martin CF. The prevention of coronary artery aneurysm in Kawasaki disease: a meta-analysis on the efficacy of aspirin and immunoglobulin treatment. Pediatrics 1995; 96:1057.  ​

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