Adults with a Pediatric Disease

First described in 1967, Kawasaki disease (KD) primarily affects children younger than 5 years, especially those of Japanese or Korean descent. Its cause is unknown, and no tests are diagnostic. Instead, its recognition depends upon the presence of 5 days or more of fever and at least four of five other criteria: 1) polymorphic exanthema; 2) erythema or indurative edema of the palms and soles during the acute phase or desquamation from the fingertips during convalescence; 3) bilateral nonsuppurative conjunctival injection; 4) oropharyngeal changes (e.g., injected or fissured lips, strawberry tongue, or injected pharynx); and 5) acute nonsuppurative cervical lymphadenopathy (>1.5 cm in diameter). The eruption varies, but most commonly it is morbilliform, erythema multiforme, or diffuse erythema and not petechial, bullous, or vesicular. The major complication is coronary artery vasculitis causing aneurysms or occlusion.

KD rarely occurs in adults, but these authors describe 10 adult cases, most of which the authors define as “incomplete KD” — an otherwise unexplained illness with 5 or more days of fever, at least two of the clinical criteria above, and laboratory evidence of inflammation (increased erythrocyte sedimentation rate or C-reactive protein). Other common features included symmetrical polyarticular arthralgias, gastrointestinal symptoms (emesis, diarrhea, abdominal pain), elevated liver enzymes, and thrombocytosis. The average age of these patients was about 28; the oldest was 68.