Oh Ji Goo, Yoon Chang Ho, Lee Chang Woo
Department of Dermatology, Hanyang University, College of Medicine, Seongdong-Gu, Seoul, Korea.
J Dermatol. 2007 Feb;34(2):135-7. doi: 10.1111/j.1346-8138.2006.00233.x.
Cowden syndrome, also known as multiple hamartoma syndrome is a rare autosomal dominant disorder characterized by multiple hamartomatous tumors of ectodermal, mesodermal and endodermal origin. A 47-year-old woman had a skin-colored plaque on the left foot. She had total abdominal hysterectomy due to uterine leiomyoma at the age of 35, and had modified radical mastectomy due to right breast cancer at 38. Thyroid adenoma was diagnosed at age 46. Physical examinations revealed macrocephaly, multiple papules of the buccal mucosa, skin tags of the neck and multiple keratotic papules of both hands. Multiple gastrointestinal polyps were detected on gastrofiberoscopy and colonoscopy. Histological examination of the skin lesion on the left foot showed an increased numbers of eccrine sweat glands and blood vessels, which are characteristic histological findings of eccrine angiomatous hamartoma (EAH), a rare benign tumor. We present a case of Cowden syndrome with an associated EAH, which has not been described in the English literatures.
考登综合征，也称为多发性错构瘤综合征，是一种罕见的常染色体显性疾病，其特征为起源于外胚层、中胚层和内胚层的多发性错构瘤性肿瘤。一名47岁女性左脚有一个肤色斑块。她35岁时因子宫平滑肌瘤接受了全腹子宫切除术，38岁时因右乳腺癌接受了改良根治性乳房切除术。46岁时诊断出甲状腺腺瘤。体格检查发现巨头症、颊黏膜多发性丘疹、颈部皮肤软垂疣和双手多发性角化丘疹。在胃镜检查和结肠镜检查中发现了多个胃肠道息肉。对左脚皮肤病变进行组织学检查显示，小汗腺和血管数量增加，这是小汗腺血管瘤性错构瘤（EAH）这一罕见良性肿瘤的特征性组织学表现。我们报告一例伴有EAH的考登综合征病例，英文文献中尚未对此进行描述。
