Department of Dermatology, Turkiye Cumhuriyeti Saglik Bakanligi Okmeydani Egitim ve Arastirma Hastanesi, Istanbul, Turkey.
Department of Dermatology, Istanbul Egitim ve Arastirma Hastanesi, Istanbul, Turkey.
Clin Exp Dermatol. 2017 Dec;42(8):906-909. doi: 10.1111/ced.13223. Epub 2017 Oct 4.
Brooke-Spiegler syndrome (BSS) is a rare, autosomal dominant disorder characterized by multiple adnexal tumours, especially trichoepitheliomas, cylindromas and occasionally spiradenomas. These lesions usually begin to appear in the second or third decade of life. Malignant transformation of pre-existing tumours may occur. In vivo reflectance-mode confocal microscopy (RCM) is a noninvasive method that can be used to visualize the epidermis and the upper dermis at almost histological resolution. It has been used to evaluate several skin conditions, especially malignant lesions, and has been reported to be useful for differentiating between malignant and benign skin tumours. Only a few studies have reported on the use of confocal microscopy to characterize the features of benign adnexal neoplasms. We present a patient with BSS who presented to our clinic with multiple skin tumours. The possible utility of RCM for identifying adnexal neoplasms is emphasized in this report, which also describes the observed microscopic features.
布鲁克-斯皮格尔综合征(BSS)是一种罕见的常染色体显性遗传病,其特征为多发性附件肿瘤,尤其是毛发上皮瘤、圆柱瘤,偶尔还有螺旋腺瘤。这些病变通常开始于生命的第二或第三个十年。先前存在的肿瘤可能发生恶性转化。体内反射模式共聚焦显微镜(RCM)是一种非侵入性方法,可用于以近乎组织学分辨率可视化表皮和上真皮。它已被用于评估几种皮肤状况,特别是恶性病变,并且据报道有助于区分恶性和良性皮肤肿瘤。只有少数研究报告了使用共聚焦显微镜来描述良性附件肿瘤的特征。我们报告了一位患有 BSS 的患者,他因多发性皮肤肿瘤就诊于我们的诊所。本报告强调了 RCM 用于识别附件肿瘤的可能效用,并描述了观察到的微观特征。