INFANTILE PERIARTERITIS NODOSA OR MUCOCUTANEOUS LYMPH NODE SYNDROME A report on four cases and diagnostic considerations

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Abstract
Coronary artery aneurysm in childhood is a rare disease and was in most cases ascribed to infantile periarteritis nodosa (IPN). In recent years a mucocutaneous lymph node syndrome (MLNS) was found almost exclusively in Japan; this disease frequently involves the coronary arteries and myocardium. Four cases with coronary aneurysms are presented from Sweden and seem to be the 1st described from Scandinavia. Three of these patients died a sudden death with cardiac arrest. Since MLNS and IPN have identical clinical and pathological features, MLNS and IPN may constitute a pathologic entity and to separate them on a clinical or histological basis is nonsensical. The risk of coronary aneurysm and possible sudden death must be considered in patients with uncharacteristic symptoms including prolonged fever, conjunctivitis, exanthema, lesions in the oral mucosa, elevated sedimentation rate and leukocytosis.

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