[Phacomatoses. Pathogenesis - Classification - Vascular aspects].
The pathogenesis of the phacomatoses, developmental diseases of the embryonic plates, permits an understanding of the different manifestations which characterize these disorders. This pathogenesis also constitutes the best basis for a rational classification. The author sets out the main features of this pathogenesis and its practical applications, and then considers the principal vascular aspects of the phacomatoses, especially in Osler-Rendu disease, Blue Rubber Bleb Naevi, Mafussi's syndrome, the haemangioblastomatoses, Bailey's glomangiomatosis, the Louis-Bar syndrome, Struge-Weber angiomatosis, the syndrome of Bonnet-Dechaume and Blanc, Cobb's syndrome, the angio-osteo-hypertrophic syndromes, von Recklinghausen's neurofibromatosis, Bourneville's tuberous sclerosis, and the melanic phacomatoses.
D000293:Adolescent; D000328:Adult; D000798:Angiomatosis / Q000145:classification / Q000196:embryology*; D005855:Germ Layers / Q000473:pathology*; D006801:Humans; D009456:Neurofibromatosis 1 / Q000145:classification / Q000196:embryology*; D009508:Nevus, Pigmented / Q000145:classification / Q000196:embryology*; D014402:Tuberous Sclerosis / Q000145:classification / Q000196:embryology*
Les phacomatoses. Pathogénie - Classification - Aspects vasculaires.