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考登病:14例新病例分析。

Cowden's disease: analysis of fourteen new cases.

作者信息

Starink T M

出版信息

J Am Acad Dermatol. 1984 Dec;11(6):1127-41. doi: 10.1016/s0190-9622(84)70270-2.

DOI:10.1016/s0190-9622(84)70270-2
PMID:6512057
Abstract

Fourteen new patients with Cowden's disease from the Netherlands were analyzed and compared with the sixty-nine previously reported patients. The full clinical spectrum of the disease can now be fairly well delineated. Mucocutaneous lesions, especially facial trichilemmomas and other follicular malformations, acral keratoses, and oral papillomas, remain the most constant findings. The incidence of each of these mucocutaneous findings was slightly higher than reported previously, probably reflecting increasing experience with this condition. Also our patients tended to have more extensive acral keratoses and perhaps less extensive facial lesions. In one family there was an extremely high incidence of lipomas. Breast cancer was seen in three of our twelve female patients, reflecting the trend toward a lower incidence of breast cancer in the recent literature. Gastrointestinal polyps in one family were much more common than previously reported, occurring in five of seven patients. Thyroid disease was found in about two thirds of the patients and abnormalities of the female reproductive system in slightly more than half of the patients, both incidences being approximately the same as in the literature. With increased awareness of the condition it is likely that many more cases will be recognized in the future.

摘要

对来自荷兰的14例新的考登病患者进行了分析,并与之前报告的69例患者进行了比较。现在可以相当清晰地勾勒出该疾病的完整临床谱系。皮肤黏膜病变,尤其是面部毛发上皮瘤和其他毛囊畸形、肢端角化病和口腔乳头状瘤,仍然是最常见的表现。这些皮肤黏膜表现的发生率均略高于之前的报告,这可能反映了对该疾病的认识不断增加。此外,我们的患者往往有更广泛的肢端角化病,面部病变可能较少。在一个家族中,脂肪瘤的发病率极高。我们的12名女性患者中有3例患有乳腺癌,这反映了近期文献中乳腺癌发病率较低的趋势。一个家族中的胃肠道息肉比之前报告的更为常见,7名患者中有5例出现。约三分之二的患者发现患有甲状腺疾病,略多于一半的患者存在女性生殖系统异常,这两种疾病的发病率与文献报道大致相同。随着对该疾病认识的提高,未来可能会发现更多病例。

相似文献

1
Cowden's disease: analysis of fourteen new cases.考登病:14例新病例分析。
J Am Acad Dermatol. 1984 Dec;11(6):1127-41. doi: 10.1016/s0190-9622(84)70270-2.
2
Cowden's disease: a cutaneous marker of breast cancer.考登病:乳腺癌的一种皮肤标志物。
Cancer. 1978 Jun;41(6):2393-8. doi: 10.1002/1097-0142(197806)41:6<2393::aid-cncr2820410644>3.0.co;2-k.
3
[Cowden's disease : multiple hamartomata syndrome (author's transl)].
Rev Stomatol Chir Maxillofac. 1979;80(5):257-9.
4
[Cowden's disease. A report on two cases in Swiss families (author's transl)].
Rev Stomatol Chir Maxillofac. 1979;80(5):246-56.
5
Disseminated hereditary gastrointestinal polyposis with orocutaneous hamartomatosis (Cowden's disease).伴有口皮肤错构瘤病的播散性遗传性胃肠息肉病(考登病)。
Endoscopy. 1984 Mar;16(2):59-63. doi: 10.1055/s-2007-1018534.
6
[Cowden's disease. Syndrome of multiple hamartomas].
J Radiol. 1984 Oct;65(10):701-4.
7
Cowden's disease (multiple hamartoma and neoplasia syndrome). A case report and review of the English literature.
J Am Acad Dermatol. 1983 May;8(5):686-96. doi: 10.1016/s0190-9622(83)70081-2.
8
Cowden's disease.考登病
Ophthalmology. 1988 Aug;95(8):1038-41. doi: 10.1016/s0161-6420(88)33066-6.
9
Cowden syndrome (multiple hamartoma syndrome).考登综合征(多发性错构瘤综合征)。
Dermatol Clin. 1995 Jan;13(1):27-31.
10
Cowden's disease (multiple hamartoma syndrome).考登病(多发性错构瘤综合征)。
Mayo Clin Proc. 1981 Mar;56(3):179-84.

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Ophthalmological manifestations in a cohort of Cowden syndrome patients in a large tertiary healthcare system.大型三级医疗保健系统中一组考登综合征患者的眼科表现。
Eye (Lond). 2025 Sep 11. doi: 10.1038/s41433-025-03966-0.
2
Orofacial Manifestations Assisting the Diagnosis of Cowden Syndrome in a Middle-Aged Patient: Case Report and Literature Overview.口面部表现辅助诊断中年患者的考登综合征:病例报告与文献综述
Head Neck Pathol. 2022 Mar;16(1):304-313. doi: 10.1007/s12105-021-01345-1. Epub 2021 Jun 9.
3
Neuroimaging abnormalities in patients with Cowden syndrome: Retrospective single-center study.
考登综合征患者的神经影像学异常:回顾性单中心研究
Neurol Clin Pract. 2018 Jun;8(3):207-213. doi: 10.1212/CPJ.0000000000000463.
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Inherited skin tumour syndromes.遗传性皮肤肿瘤综合征。
Clin Med (Lond). 2017 Dec;17(6):562-567. doi: 10.7861/clinmedicine.17-6-562.
5
Multiple Meningiomas in a Patient with Cowden Syndrome.考登综合征患者的多发性脑膜瘤
J Neurol Surg Rep. 2016 Jul;77(3):e128-33. doi: 10.1055/s-0036-1584265.
6
Benign follicular tumors.良性滤泡性肿瘤
An Bras Dermatol. 2015 Nov-Dec;90(6):780-96; quiz 797-8. doi: 10.1590/abd1806-4841.20154114.
7
AKT activation promotes PTEN hamartoma tumor syndrome-associated cataract development.AKT 激活促进了 PTEN 错构瘤肿瘤综合征相关白内障的发展。
J Clin Invest. 2013 Dec;123(12):5401-9. doi: 10.1172/JCI70437. Epub 2013 Nov 25.
8
The skin: a mirror to the gut.皮肤:肠道的一面镜子。
Int J Colorectal Dis. 2013 Jul;28(7):889-913. doi: 10.1007/s00384-012-1637-x. Epub 2013 Jan 18.
9
Acinic cell carcinoma of the retromolar trigone region: expanding the tumor phenotype in Cowden syndrome?磨牙后三角区的腺泡细胞癌:Cowden 综合征中肿瘤表型的扩展?
Fam Cancer. 2011 Dec;10(4):691-4. doi: 10.1007/s10689-011-9472-8.
10
Cowden syndrome: a critical review of the clinical literature.考登综合征:临床文献的批判性综述
J Genet Couns. 2009 Feb;18(1):13-27. doi: 10.1007/s10897-008-9187-7. Epub 2008 Oct 30.