Occurs primarily in children within the first
years of life. Main clinical signs are fever, conjunctivitis, inflammation of
the mucous membranes and tongue (strawberry tongue),
swollen erythematous hands and feet,
truncal rashes, and diffuse lymphadenopathy. Cardiac complications include
pericarditis, myocarditis, myocardial infarction, or ischemia as a result of
coronary artery thrombosis or stenosis, and valvular dysfunction.
Pneumonitis and pleural effusions can occur. Cerebral hemorrhage caused by
aneurysm rupture has been reported. Other CNS complications are cranial nerve palsies, aseptic
meningitis, and subdural effusions. Aneurysmal rupture of any affected
vessel present with hemorrhage. Stenosis of vessels can cause
hypoperfusion of distal extremities or other organs. Hepatic involvement
occurs in less than 10% of cases, and renal involvement occurs in less
than 1%. Anemia may be present. Management consists of γ-globulin
and aspirin.
Corticosteroids, pentoxifylline (a methyl-xanthine compound that inhibits TNF-α
messenger RNA-transcription), ulinastatin (a human trypsin inhibitor that inhibits neutrophil
elastase), abciximab (a platelet glycoprotein IIb/IIIa receptor antagonist),
monoclonal antibodies, and cytotoxic agents (cyclophosphamide) have
all been used with variable success in these patients.