Pujol R M, Ravella A, Noguera X, de Moragas J M, Moreno A
Servicio de Dermatología, Hospital San Pablo, Barcelona.
Med Cutan Ibero Lat Am. 1988;16(4):322-7.
We describe the clinicopathological features of multiple hamartoma and neoplasia syndrome (Cowden's syndrome) in a 58 year-old man and in a 56 year-old woman. The typical mucocutaneous lesions of this disorder were present in both cases. Visceral hamartomas involving thyroid and gastrointestinal system (polyposis) were present in both patients. Dermal or submucous fibromas are proposed as an additional diagnostic criterium of Cowden's syndrome. Associated abnormalities and visceral involvement in multiple hamartoma syndrome are reviewed.
我们描述了一名58岁男性和一名56岁女性的多发性错构瘤和肿瘤综合征(考登综合征)的临床病理特征。这两名患者均出现了该疾病典型的黏膜皮肤病变。两名患者均存在累及甲状腺和胃肠道系统的内脏错构瘤(息肉病)。皮肤或黏膜下纤维瘤被提议作为考登综合征的一项附加诊断标准。本文对多发性错构瘤综合征相关的异常情况和内脏受累情况进行了综述。