Lee H R, Moon Y S, Yeom C H, Kim K W, Chun J Y, Kim H K, Choi H S, Kim D K, Chung T S
Department of Family Medicine, Yonsei University College of Medicine, Seoul, Korea.
J Korean Med Sci. 1997 Dec;12(6):570-5. doi: 10.3346/jkms.1997.12.6.570.
Cowden's disease, or multiple hamartoma syndrome, is an uncommon condition with characteristic mucocutaneous lesions associated with abnormalities of the breast, thyroid, and gastrointestinal tract. We describe a 32-year-old man with oral mucosal papillomatosis and plantar hyperkeratosis as a definite case of Cowden's disease according to the criteria proposed by Salem and Steck. The patient also had a thyroid mass and numerous gastrointestinal polyps endoscopically. Histologically the polyps were hamartomatous or hyperplastic polyps. The oral papillary lesions were fibroepithelial polyps and the thyroid mass was a follicular adenoma. We review the literature on this entity and summarize the pertinent findings. To the best of our knowledge, this is the first documented case of Cowden's disease in a Korean.
考登病,又称多发性错构瘤综合征,是一种罕见疾病,具有特征性的黏膜皮肤病变,与乳腺、甲状腺及胃肠道异常相关。我们描述一名32岁男性,根据塞勒姆和斯特克提出的标准,该患者患有口腔黏膜乳头状瘤病和足底角化过度,确诊为考登病。该患者还在内镜检查中发现有甲状腺肿块及众多胃肠道息肉。组织学检查显示,息肉为错构瘤性或增生性息肉。口腔乳头状病变为纤维上皮性息肉,甲状腺肿块为滤泡性腺瘤。我们回顾了关于该疾病的文献并总结了相关发现。据我们所知,这是韩国首例有文献记载的考登病病例。