[Mucocutaneous lymph node syndrome in Austria. Four cases with one fatal outcome. 2. Pathological findings].
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Резюме
First pathoanatomic case report of mucocutaneous lymph-node syndrome (MCLS) from Austria: A 3 1/2 year old boy was admitted to the Pediatric Department with a six day history of fever and bilateral conjunctivitis. He showed a maculous exanthema, red and fissuring lips, reddened tonsils and a coated tongue. Submandibular lymph nodes were swollen (1 cm diameter); ESR 128/138; peripheral leukocytosis 17 x 10(9)/l. On the tenth day of illness membranous desquamation from the fingertips and a strawberry tongue was observed. The ECG showed a myocardial infarction in the apical portion of the left ventricle. The boy died on the 25th day of illness. Pathological findings were found predominantly in the heart: Coronary arteries showed fibrous thickening of intimal layer, focal destruction of elastic membrane and aneurysms in both arteries. The aneurysm in the descending branch of the left coronary artery was 15 mm long and had a maximum diameter of 8 mm. It was completely occluded by a thrombus. Recurring anteroseptal myocardial infarction. Other investigated arteries were not affected. Tonsils as well as (paratracheal and parapancreatic) lymph-nodes showed a few ceroid pigment containing histiocytes.