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单侧节段性排列的基底细胞样滤泡性错构瘤伴发皮肤骨瘤和缺牙:一例 Happle-Tinschert 综合征。

Unilateral segmentally arranged basaloid follicular hamartomas with osteoma cutis and hypodontia: a case of Happle-Tinschert syndrome.

机构信息

Department of Dermatology, Chang Gung Memorial Hospital, Keelung, Taiwan; Chang Gung University College of Medicine, Taoyuan, Taiwan.

出版信息

Clin Exp Dermatol. 2013 Dec;38(8):862-5. doi: 10.1111/ced.12161.

Abstract

Happle-Tinschert syndrome (HTS) is a rare syndrome characterized by segmentally arranged basaloid follicular hamartomas (BFH) associated with ipsilateral osseous, dental and cerebral abnormalities. Happle and Tinschert first reported this disorder in 2008, and three cases with similar presentations have since been reported. We report another case, that of a 40-year-old man, presenting with the characteristic clinical features of HTS.

摘要

哈普尔-廷谢尔特综合征(HTS)是一种罕见的综合征,其特征为节段性排列的基底细胞滤泡错构瘤(BFH),伴有同侧骨、牙和脑的异常。Happle 和 Tinschert 于 2008 年首次报道了这种疾病,此后又有三例类似表现的病例报告。我们报告了另一个病例,一个 40 岁的男性,具有 HTS 的典型临床特征。

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