Kawasaki Disease is typically a self-limiting, medium vessel vasculitis that predominantly affects Asian male children between the ages of 1 to 5.1 One of the major complications from Kawasaki Disease is the development of coronary artery aneurysms. If left untreated, 20-25% of the cases of Kawasaki Disease are associated with coronary artery aneurysms,2 but the risk is reduced to <5% when treated appropriately.3 Thus, it is important to recognize the presentation of Kawasaki Disease in the emergency room setting.
When thinking about the diagnostic criteria for Kawasaki Disease, think of the mnemonic WARM CREAM
Warm – 5 days of fever or more
Plus 4 of 5:
- Conjunctivitis – bilateral, non-purulent, peri-limbic sparing
- Rash – generalized non-vesicular (commonly maculopapular, morbilliform of the trunk but no specific rash is pathognomonic)
- Extremity changes – erythema and edema of hands and feet, followed by desquamation
- Adenopathy – anterior cervical with at least one palpable node ≥1.5 cm
- Mucous membrane changes – cracked red lips, “strawberry” tongue
Treatment is IVIG (2 g/kg) and ASA (dose is institution specific) to prevent cardiovascular complications.2,3 An echocardiogram should be obtained at baseline and in 2-6 weeks for monitoring any potential disease progression.
This post was edited by Daniel Ting.
This post was copyedited by Jeremi Laski
- 1.Newburger JW, Takahashi M, Burns JC. Kawasaki Disease. Journal of the American College of Cardiology. Published online April 2016:1738-1749. doi:10.1016/j.jacc.2015.12.073
- 2.Langford C, Fauci A. The Vasculitis Syndromes. In: Harrison’s Principles of Internal Medicine. 20th ed. McGraw-Hill Education; 2019:2588.
- 3.McCrindle B, Rowley A, Newburger J, et al. Diagnosis, Treatment, and Long-Term Management of Kawasaki Disease: A Scientific Statement for Health Professionals From the American Heart Association. Circulation. 2017;135(17):e927-e999. doi:10.1161/CIR.0000000000000484
Reviewing with the Staff
While Kawasaki disease is relatively uncommon, the morbidity it confers when untreated is high with respect to heart disease in young children. Kawasaki disease should be on the differential diagnosis for any child presenting to the emergency department with prolonged fever. In addition to using the WARM CREAM mnemonic, laboratory and diagnostic investigations assist in verifying the diagnosis. Where there is a high index of suspicion but not all the criteria are met, patients should be referred to a pediatric team/facility to investigate and treat potential incomplete Kawasaki disease.