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58例结节性硬化症成年患者的口腔检查结果

Oral findings in 58 adults with tuberous sclerosis complex.

作者信息

Sparling Joshua D, Hong Chien-Hui, Brahim Jaime S, Moss Joel, Darling Thomas N

机构信息

National Capital Consortium Residency Program, Walter Reed Army Medical Center, Washington, DC, USA.

出版信息

J Am Acad Dermatol. 2007 May;56(5):786-90. doi: 10.1016/j.jaad.2006.11.019. Epub 2007 Jan 19.

Abstract

BACKGROUND

Gingival fibromas and dental pitting are among the diagnostic criteria for tuberous sclerosis complex (TSC).

OBJECTIVE

Our goal was to document the oral findings in 58 adult patients with TSC.

RESULTS

Forty patients (69%) had oral fibromas, appearing mostly on the attached or interdental gingiva. Other oral mucosal sites with fibromas included buccal and labial mucosa, the superior labial frenulum, palate, and tongue. In all, 56 patients (97%) had multiple dental enamel pits.

LIMITATIONS

This case series comprised predominantly adult women with TSC and lymphangioleiomyomatosis.

CONCLUSIONS

Oral fibromas in TSC are mostly, but not exclusively, gingival. Dental pits are present in nearly all patients. The multiple oral papules in TSC may appear similar to those observed in Cowden syndrome, Birt-Hogg-Dubé syndrome, and rarely in multiple endocrine neoplasia type 1.

摘要

背景

牙龈纤维瘤和牙面凹坑是结节性硬化症(TSC)的诊断标准之一。

目的

我们的目标是记录58例成年TSC患者的口腔检查结果。

结果

40例患者(69%)有口腔纤维瘤,主要出现在附着龈或牙间龈。出现纤维瘤的其他口腔黏膜部位包括颊黏膜、唇黏膜、上唇系带、腭部和舌部。共有56例患者(97%)有多个牙釉质凹坑。

局限性

该病例系列主要包括患有TSC和淋巴管平滑肌瘤病的成年女性。

结论

TSC患者的口腔纤维瘤大多(但并非全部)出现在牙龈。几乎所有患者都有牙面凹坑。TSC中的多个口腔丘疹可能与考登综合征、Birt-Hogg-Dubé综合征中观察到的相似,在1型多发性内分泌肿瘤中则很少见。

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