Cowden's disease.

Cowden's disease.
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考登氏病。

DOI:
10.1016/s0161-6420(88)33066-6
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发表时间:
1988
期刊:
影响因子:
13.7
通讯作者:
Boatwright,RS
Boatwright,RS
中科院分区:
医学1区
文献类型:
--
作者:
Bardenstein,DS;McLean,IW;Nerney,J;Boatwright,RS

文献摘要

被引文献

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柯登氏病,或多发性错构瘤综合征,是一种罕见的遗传性皮肤病。多发性面部毛鞘瘤、肢端角化病和口腔乳头状瘤是最常见的皮肤粘膜病变。它与乳腺癌和甲状腺癌以及多器官系统的良性肿瘤和错构瘤的发病率增加有关。多发性面部毛鞘瘤是这种综合征的主要特征,可能表现为眼睑和眶周皮肤的病变。一例柯登氏病的描述和以前报道的情况下审查,重点是眼部方面的疾病。
Cowden's disease, or multiple hamartoma syndrome, is a rare genodermatosis. Multiple facial trichilemmomas, acral keratoses, and oral papillomas are the most common mucocutaneous lesions in this disease. It is associated with increased rates of cancer of the breast and thyroid as well as benign tumors and hamartomas of multiple organ systems. Multiple facial trichilemmomas are essentially pathognomonic of this syndrome and may appear as lesions of the eyelid and periorbital skin. A case of Cowden's disease is described and previously reported cases reviewed, with emphasis on the ocular aspects of the disease.