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遗传性多发性纤维毛囊瘤、毛发上皮瘤和皮赘:Birt-Hogg-Dubè综合征。

Hereditary multiple fibrofolliculomas, trichodiscomas and acrochordons: syndrome of Birt-Hogg-Dubè.

作者信息

Scalvenzi M, Argenziano G, Sammarco E, Delfino M

机构信息

Department of Dermatology, University of Naples Federico II, Italy.

出版信息

J Eur Acad Dermatol Venereol. 1998 Jul;11(1):45-7.

PMID:9731965
Abstract

The syndrome of Birt-Hogg-Dubè (BHD) is an autosomal dominant syndrome, characterized by a triad of cutaneous lesions including multiple fibrofolliculomas, trichodiscomas, and acrochordons. There are many clinical expressions, solitary and multiple forms, with or without other skin tumors. In the literature BHD syndrome has been associated with internal malignancy. We describe a patient with multiple firm, skin-colored papules in which the three types of lesion are documented. No signs of systemic disease were present.

摘要

Birt-Hogg-Dubè(BHD)综合征是一种常染色体显性综合征,其特征为三联征皮肤损害,包括多发性纤维毛囊瘤、毛发上皮瘤和皮赘。它有多种临床表现形式,有单发和多发形式,伴或不伴有其他皮肤肿瘤。在文献中,BHD综合征与内脏恶性肿瘤有关。我们描述了一位患者,其有多个质地坚实、肤色丘疹,且记录到了三种类型的损害。未发现全身性疾病的体征。

相似文献

1
Hereditary multiple fibrofolliculomas, trichodiscomas and acrochordons: syndrome of Birt-Hogg-Dubè.遗传性多发性纤维毛囊瘤、毛发上皮瘤和皮赘:Birt-Hogg-Dubè综合征。
J Eur Acad Dermatol Venereol. 1998 Jul;11(1):45-7.
2
[Fibrofolliculoma, trichodiscoma and acrochordon. The Birt-Hogg-Dubé syndrome].[纤维毛囊瘤、毛发上皮瘤和皮赘。Birt-Hogg-Dubé综合征]
Ann Dermatol Venereol. 1983;110(8):601-9.
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Familial multiple trichodiscomas. A clinicopathologic study.家族性多发性毛发盘状瘤。一项临床病理研究。
Arch Dermatol. 1985 Jul;121(7):888-91.
4
Acrochordons are not a component of the Birt-Hogg-Dubé syndrome: does this syndrome exist? Case reports and review of the literature.皮赘并非Birt-Hogg-Dubé综合征的组成部分:该综合征是否存在?病例报告及文献综述
Am J Dermatopathol. 1999 Aug;21(4):369-74. doi: 10.1097/00000372-199908000-00011.
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Birt-Hogg-Dubé Syndrome.Birt-Hogg-Dubé综合征。
Int J Dermatol. 2005 Aug;44(8):668-73. doi: 10.1111/j.1365-4632.2004.02095.x.
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Fibrofolliculoma in a patient with tuberous sclerosis complex.结节性硬化症患者的纤维毛囊瘤。
Clin Exp Dermatol. 2009 Dec;34(8):892-4. doi: 10.1111/j.1365-2230.2008.03065.x. Epub 2009 Jan 15.
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Localized Birt-Hogg-Dubé syndrome with prominent perivascular fibromas.伴有显著血管周围纤维瘤的局限性Birt-Hogg-Dubé综合征
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Detection of 1733insC mutations in an Asian family with Birt-Hogg-Dubé syndrome.一个患Birt-Hogg-Dubé综合征的亚洲家族中1733insC突变的检测。
Br J Dermatol. 2005 Jan;152(1):142-5. doi: 10.1111/j.1365-2133.2004.06283.x.
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Parotid oncocytoma in the Birt-Hogg-Dubé syndrome.
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[Sporadic Birt-Hogg-Dube syndrome].
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Multiple/bilateral renal tumors in patients with Birt-Hogg-Dubé syndrome.
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