[Sarcomatous degeneration of a familial cavernous angiomatosis (Bean's syndrome) (author's transl)].
Although the classification of "Ullman's universal angiomatosis" has not yet been completely defined, the authors estimed it interesting to report an observation which may fall into this denomination. This observation concerns a generalized cavernous angiomatosis differing from hereditary hemorrhagic telangiectasia (Rendu-Osler type) with its conventional skin and mucous membrane manifestations and its visceral forms. The patient presented cutaneous and probably digestive generalized cavernomatosis related to blue rubber bleb naevus. In connection with this observation, the authors attempt to offer reasons in support of the distinctions which appear to exist between glomangiomatosis and glomangioma. This observation is also interesting from two other standpoints: 1. the striking lineal descent: in five generations, seven subjects presented identical angiomatoses; 2. the malignant transformation (angiosarcoma) of one of the cavernous elements.
D000368:Aged; D000798:Angiomatosis / Q000235:genetics* / Q000473:pathology; D003937:Diagnosis, Differential; D005260:Female; D006392:Hemangioma, Cavernous / Q000235:genetics* / Q000473:pathology; D006394:Hemangiosarcoma / Q000473:pathology*; D006801:Humans; D009378:Neoplasms, Multiple Primary / Q000473:pathology*; D010375:Pedigree; D012878:Skin Neoplasms / Q000235:genetics* / Q000473:pathology; D013577:Syndrome
Dégénérescence sarcomateuse d'une angiomatose caverneuse familiale (syndrome de Bean).