Kawasaki disease (KD; also known as mucocutaneous lymph node syndrome) is an immune-mediated disorder of unknown etiology that occurs in genetically predisposed children leading to endothelial cell injury and vasculitis of mainly medium-sized arteries (predilection for coronary arteries). Small arterioles, larger arteries, capillaries, and veins are affected to a lesser extent. KD occurs in all ethnic groups; however, incidence is highest in East Asia and in children of Asian descent. Clinical and epidemiologic features strongly support an infectious etiology. About 80% of patients are <5 years of age (peak: between 18 and 24 months); about 90% of patients are <8 years. There is no specific test for KD; diagnosis is based on clinical criteria summarized in Table 12.1. Other findings include extreme irritability, aseptic meningitis (50%), urethritis (sterile pyuria, 70%), hepatic dysfunction (40%), hydrops of gallbladder, diarrhea, vomiting, abdominal pain, arthritis, or arthralgia (knees, ankles, hips), uveitis, pneumonitis, testicular swelling, peripheral gangrene, erythema, or induration at bacille Calmette-Guérin inoculation site.
Table 12.1 – Clinical Features of Kawasaki Disease |Favorite Table|Download (.pdf)
Table 12.1 – Clinical Features of Kawasaki Disease
|Diagnosis is established clinically by:|
Presence of fever and at least 4 of the 5 criteria listed below, or
Presence of fever and at least 3 of the 5 criteria listed below, and evidence of coronary artery abnormalities
Fever: high and unremitting (lasting 5 days or more, unresponsive to antipyretics and antibiotics)
Skin rash: polymorphic (morbilliform, maculopapular, scarlatiniform, or erythema multiforme–like); nonvesicular; commonly seen on trunk and extremities
Mucous membrane changes (at least one of the following): erythematous or fissured lips; erythema of buccal mucosa and pharynx; “strawberry” tongue
Conjunctivitis: bilateral bulbar involvement; nonexudative
Changes in distal extremities (at least one of the following): erythema of palms or soles; indurative edema of hands or feet; periungual desquamation of fingers and toes (1–3 weeks after onset of illness)
Cervical lymphadenopathy (least constant finding): unilateral; at least one node 1.5 cm or larger in diameter; nonpurulent
Clinical phases (after onset of illness):
- Acute febrile phase: up to 1 to 2 weeks (fever and other acute signs)
- Subacute phase: up to 2 to 4 weeks (fever abated, desquamation, thrombocytosis, development of coronary aneurysms)
- Convalescent phase: up to 6 to 8 weeks (illness disappears, ESR, C-reactive protein return to normal)
Figure 12.1 ▪ Kawasaki Disease.
A 3-year-old child with a history of high fever of 6 days’ duration associated with diffuse erythematous maculopapular rash, red lips, and bilateral conjunctival injection. (Photo contributor: Binita R. Shah, MD.)
Figure 12.2 ▪ Features of Kawasaki Disease (KD).
Log In to View More
If you don't have a subscription, please view our individual subscription options below to find out how you can gain access to this content.
Want remote access to your institution's subscription?
Sign in to your MyAccess profile while you are actively authenticated on this site via your institution (you will be able to verify this by looking at the top right corner of the screen - if you see your institution's name, you are authenticated). Once logged in to your MyAccess profile, you will be able to access your institution's subscription for 90 days from any location. You must be logged in while authenticated at least once every 90 days to maintain this remote access.
If your institution subscribes to this resource, and you don't have a MyAccess profile, please contact your library's reference desk for information on how to gain access to this resource from off-campus.
AccessPediatrics Full Site: One-Year Subscription
Connect to the full suite of AccessPediatrics content and resources including 20+ textbooks such as Rudolph’s Pediatrics and The Pediatric Practice series, high-quality procedural videos, images, and animations, interactive board review, an integrated pediatric drug database, and more.
Pay Per View: Timed Access to all of AccessPediatrics
24 Hour Subscription $34.95
48 Hour Subscription $54.95
Pop-up div Successfully Displayed
This div only appears when the trigger link is hovered over.
Otherwise it is hidden from view.