Mukokutanes Lymphknotensyndrom in Österreich - Vier Fälle mit einem letalen Ausgang
2. Teil: Pathomorphologische Befunde*

Abstract

First pathoanatomic case report of mucocutaneus lymphnode 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 conjunctivits. He showed a maculous exanthema, red and Assuring lips, reddened tonsils and a coated tongue. Submandibular lymphnodes were swollen (1 cm diameter); ESR 128/138; peripheral leukoytosis 17 × 10⁹/1. 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: Coronaryarteries showed fibrous thickening of intimal layer, focal desctruction 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.

Zusammenfassung

Fallbericht eines 3 1/2 Jahre alten Knaben (Eltern Landwirte in Österreich), der klinisch das typische Bild eines mukokutanen Lymphknotensyndrome (MCLS) bot und am 25. Krankheitstag verstarb. Pathoanatomisch fanden sich Aneurysmen beider Coronararterien mit partieller Thrombose und einem rezidivierenden anteroseptalen Myokardinfarkt. Pathohistologisch war der Prozeß in den Coronarien durch verschiedene Stadien der Thrombose, Endarteriitis und Mediazerstörung gekennzeichnet. Das Bild einer klassischen Panarteriitis lag nicht vor. Andere untersuchte Arterien waren nicht befallen.