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家族性海绵状血管瘤病(Bean综合征)的肉瘤样变性(作者译)

[Sarcomatous degeneration of a familial cavernous angiomatosis (Bean's syndrome) (author's transl)].

作者信息

Sarrat L, Sarrat P

出版信息

Ann Dermatol Venereol. 1980 Apr;107(4):259-65.

PMID:7189993
Abstract

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.

摘要

尽管“厄尔曼全身性血管瘤病”的分类尚未完全明确,但作者认为报告一个可能属于该命名的病例很有意思。该病例涉及一种全身性海绵状血管瘤病,与遗传性出血性毛细血管扩张症(伦杜 - 奥斯勒型)不同,后者有其典型的皮肤和黏膜表现以及内脏型表现。患者表现出与蓝色橡皮疱痣综合征相关的皮肤及可能累及消化道的全身性海绵状血管瘤病。结合该病例,作者试图给出理由,以支持血管球瘤病和血管球瘤之间似乎存在的区别。从另外两个角度来看,该病例也很有意思:1. 显著的直系遗传:在五代人中,有七名受试者患有相同的血管瘤病;2. 其中一个海绵状病变发生了恶性转化(血管肉瘤)。

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