Abstract
Nine children were seen with an unusual and highly distinctive symptom complex consisting of high fever for > 7 days not responding to antibiotics; striking polymorphous exanthem, either morbilliform, scarlatiniform or multiforme; marked erythema of palms and soles with severe indurative edema progressing to desquamation of digits; stomatitis with fissuring of lips, diffuse mucosal erythema and strawberry tongue; conjunctivitis; and polymorphonuclear leukocytosis. Eight patients had associated cervical lymphadenopathy, 4 acute urethritis, 3 arthritis, 3 diarrhea and 2 aseptic meningitis. One child died suddenly on day 21; bilateral coronary artery thrombosis, extensive myocardial infarction, aortitis, localized arteritis and meningeal inflammation were demonstrated. Viral and bacterial studies of all patients were negative. Only 1 child developed ASO titer elevation. Children were < 8 years, of both sexes and multiple races.
Patients with this unusual multisystem disease have a predictable course and resemble each other more than they resemble any syndrome recognized in the U.S. Their clinical course is indistinguishable from MCLS or Kawasaki disease widely recognized in Japan where > 2200 cases have been seen. Mortality rate is 1.3% from coronary artery thrombosis, Despite extensive investigation in Japan the etiology is unknown. Recognition of these cases in the U.S. demonstrates the need for wider awareness of this significant, distinctive disease.
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Melish, M., Hicks, R., Larson, E. et al. MUCOCUTANEOUS LYMPH NODE SYNDROME (MCLS) IN THE U.S. Pediatr Res 8, 427 (1974). https://doi.org/10.1203/00006450-197404000-00523
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DOI: https://doi.org/10.1203/00006450-197404000-00523
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