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Dermatologic Manifestations of Kawasaki Disease

Overview

Kawasaki disease is a dynamic vasculitic disorder that presents with various mucocutaneous findings, which are most pronounced at different times. (See the chart below.) The most characteristic features of Kawasaki disease are usually present at the time of presentation, or appear shortly thereafter.

Clinical manifestations and time course of Kawasak

Clinical manifestations and time course of Kawasaki disease. Courtesy of Paul R. Ogershok, MD.

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The five major clinical findings in Kawasaki disease are as follows
:

Change in the extremities, typically painful erythema and edema

Polymorphous exanthem

Changes in the lips and oropharyngeal mucosa

Bilateral, nonexudative, bulbar conjunctival injection

Unilateral, nonsuppurative cervical lymphadenopathy

See Kawasaki Disease: Do You Know the Signs?, a Critical Images slideshow, to help identify the specific criteria for diagnosis.

Treatment summary

The goal of treatment is to reduce inflammation in walls of the coronary arteries in order to decrease the risk of coronary thrombosis and other cardiac complications. Although the standard of care of Kawasaki disease is intervenous immunoglobulin (IVIG)
and high-dose aspirin, approximately 15-20% of children are nonresponders (having persistent or recurrent fever after therapy); these patients have higher risk for coronary artery damage and require additional therapy.
In Japan, the Egami score (based on age, duration of illness, platelet count, and C-reactive protein) is a sensitive and specific tool used at diagnosis to determine IVIG treatment resistance.
Several large randomized trials have been performed, which have shown reduced coronary abnormalities when intravenous methylprednisolone-pulse (IVMP) was added to traditional IVIG in patients with severe or refractory Kawasaki disease.

However, it is important to keep in mind that Kawasaki disease is not a homogeneous disease, even in Japan, and the Egami score has not been well studied for non-Japanese populations. So at this juncture, IVMP plus IVIG therapy appears safe and effective for Kawasaki disease patients who are deemed to have refractory Kawasaki disease based on Egami score, at least in the Japanese population.

Other treatments that have been used for IVIG-resistant patients include additional does of IVIG; Ulinastatin (human trypsin inhibitor), which is primarily used in Japan; plasma exchange; immunosuppressants; and, recently, tumor necrosis factor-α inhibitor (infliximab).

Go to Kawasaki Disease and Ophthalmologic Manifestations of Kawasaki Disease for complete information on these topics.

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