A 2-year-old boy has been ill with fever for almost 1 week

intravenous immunoglobulin (IVIG) and aspirin

Educational Objective:

Recognize the manifestations of and treat Kawasaki disease.

Key Point:

Kawasaki disease is a vasculitis with an unclear cause that is diagnosed based on clinical symptoms and is treated with IVIG and aspirin, with the goal of preventing coronary artery aneurysms or thrombosis.

Explanation:

This child has Kawasaki disease, a vasculitis of medium-sized arteries that is associated with coronary artery–related complications, including aneurysms and thrombosis. The diagnosis is clinical, based on febrile children having a combination of the following:

• Nonexudative conjunctival injection with limbal sparing
• Tongue and lip erythema and pharyngeal erythema
• Cervical lymphadenopathy
• Swelling and/or rash of the extremities
• Polymorphous rash

Laboratory findings are nonspecific and often include leukocytosis, thrombocytosis, and elevated inflammatory markers. Treatment is with IVIG and aspirin.

Steroids have not been found to be helpful in most cases. The fever of Kawasaki disease is often unresponsive to acetaminophen. Penicillin would not be helpful in the absence of bacterial infection.

References:

McCrindle BW, Rowley AH, Newburger JW, 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:e927.

Son MB, Newburger J. Kawasaki disease (chapter 166). In: Kliegman RM, et al. Nelson Textbook of Pediatrics. 20th ed., 2016:1209-1214.