“ACUTE FEBRILE ERYTHEMATOUS SYNDROME” – T KAWASAKI, 1967

A child presents with a fever and systemic constitutional symptoms. Could this be Kawasaki disease?

 

Many of the diagnostic features of Kawasaki disease are nonspecific, and indeed, in one study 46% of patients who met criteria for Kawasaki disease were actually found to have a different disease.(Burns J Pediatrics 1991)

 

Kawasaki disease is a systemic vasculitis of medium sized arteries.(Takahashi Clin Exp Immunol 2011) Coronary arteritis begins day 6-8 and the monocyte/macrophage damage continues for several weeks.

 

The first alert is prolonged fever. In the first few days of illness the disease is often not distinguishable from many viral illnesses. After five days of fever, Kawsaki disease should be considered.(Newburger Circulation 2004)

 

Consider the original case description. Tomisaku Kawasaki introduced the disease in 1967, describing it as an “acute febrile erythematous syndrome.”(Kawasaki Pediatric Infect Dis J 2002, translation)

 

Erythema affects the eyes, lips, and oral mucosa. The conjunctivitis is classically limbus-sparing, meaning a halo of white sclera surrounds the iris. The erythema of the lips and oral mucosa is deep and colorful. Kawasaki writes “in our cases we noted only dryness, redness, erosion and cracking of lips and diffuse injection of whole oral mucosal membrane, which never formed aphthae, ulcers or pseudomembrane.”

 

As for the other classic signs, they are either nonspecific (cervical lymphadenopathy) or occur late, after we would like to have already made the diagnosis (desquamation). Focus on the erythema and the fever as means of “triggering” you to consider the diagnosis.

 

TAKE HOME POINTS:

Prolonged fever prompts the clinician to consider Kawasaki disease

The vasculitis produces a deep erythema of the conjunctivae and lips/oral mucosa.

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