[Micronodular generalized familial angiomatosis (generalized essential telangiectasia): clinical cases; ultrastructural study].
Six patients of the same family present with micronodular and generalized familial angiomatosis. Four of them have been investigated. Their problem is purely esthetic; however an asymptomatic form of von Willebrand disease has been found in a female and one of her daughters. The light microscope reveals a network of dilated capillaries in the superficial dermis. Electron microscope investigation of the endothelium demonstrates on abundance of Weibel-Palade bodies, the presence of osmiophilic inclusions within clear vacuoles, the occurrence of long spacing collagen fibrils in the vicinity of endothelial and perithelial cells; furthermore, cytoplasmic projections within the lumen constitute the most dramatic and constant feature: there are many villosities, loops, coils, tufts and entanglements in all four cases.
Bourlond
A
A
Service de Dermatologie, Faculté de Médecine, Cliniques universitaires, Bruxelles.
Minet
A
A
Marcoux
C
C
Frankart
M
M
de Sélys
R
R
D000293:Adolescent; D000328:Adult; D000798:Angiomatosis / Q000235:genetics* / Q000648:ultrastructure; D004730:Endothelium, Vascular / Q000648:ultrastructure; D005260:Female; D006801:Humans; D010375:Pedigree; D012878:Skin Neoplasms / Q000235:genetics* / Q000648:ultrastructure; D013684:Telangiectasis / Q000235:genetics* / Q000473:pathology; D014842:von Willebrand Diseases / Q000150:complications
Angiomatose familiale micronodulaire généralisée. (télangiectasies essentielles généralisées): observations cliniques; étude ultrastructurale.