[Syringomas mongolism, Marfan's disease and Ehlers-Danlos' disease].
The authors report a quite unusual case in which an eruption of syringomas, mongolism, Marfan's disease and Ehlers-Danlos' disease were associated. They emphasize the originality of the intricate appearance of syringomas on the ichthyosiform skin due to mongolism. They also stress the importance of the dysplasia of the connective tissue demonstrated by electron microscopy and the cause-effect relationships between the dysplasia of the connective tissue and the syringomas, the former accounting for the latter.
D000236:Adenoma / Q000150:complications*; D000328:Adult; D004314:Down Syndrome / Q000150:complications*; D004535:Ehlers-Danlos Syndrome / Q000150:complications*; D006801:Humans; D008297:Male; D008382:Marfan Syndrome / Q000150:complications*; D013544:Sweat Gland Neoplasms / Q000150:complications*
Syringomes, mongolisme, maladie de marfan et syndrome d'ehlers-danlos. Nouvelle entité posant les rapports des syringomes avec les maladies héréditaires du tissu conjonctif