Schirren C G, Wörle B, Kind P, Plewig G
Department of Dermatology, Ludwig-Maximilians-University, Munich, Germany.
J Cutan Pathol. 1995 Dec;22(6):563-9. doi: 10.1111/j.1600-0560.1995.tb01152.x.
Brooke-Spiegler syndrome is characterized by the development of multiple trichoepitheliomas and cylindromas. In addition, multiple spiradenomas have been observed in this autosomal-dominant inherited disease. We report a 53-year-old woman with multiple cylindromas on the head and neck and multiple trichoepitheliomas on the face. Additionally, she had had since birth a plaque on the right side of her neck containing two nodules with features of both cylindroma and trichoepithelioma. Immunohistochemical investigations revealed in the basaloid cells of trichoepithelioma an expression of CK 5/6, CK 14, CK 17, CK 19 and vimentin. The cells of the cylindroma lacked vimentin but expressed additionally CK 7, CK 8 and CK 18. The occurrence of cylindroma and trichoepithelioma in a single nevoid plaque from a patient with Brooke-Spiegler syndrome implies an alteration in the stem cells of the folliculosebaceous-apocrine unit and could be characteristic of the disorder.
布鲁克 - 施皮格勒综合征的特征是多发性毛发上皮瘤和圆柱瘤的发生。此外，在这种常染色体显性遗传病中还观察到多发性汗腺瘤。我们报告了一名53岁女性，其头颈部有多个圆柱瘤，面部有多个毛发上皮瘤。此外，她自出生以来颈部右侧有一个斑块，包含两个具有圆柱瘤和毛发上皮瘤特征的结节。免疫组织化学研究显示，毛发上皮瘤的基底样细胞中CK 5/6、CK 14、CK 17、CK 19和波形蛋白呈阳性表达。圆柱瘤细胞缺乏波形蛋白，但额外表达CK 7、CK 8和CK 18。来自布鲁克 - 施皮格勒综合征患者的单个痣样斑块中出现圆柱瘤和毛发上皮瘤，提示毛囊皮脂腺 - 顶泌汗腺单位干细胞发生改变，可能是该疾病的特征。
