Department of Oncologic Dermatology, San Gallicano Dermatologic Institute, Rome, Italy.
J Eur Acad Dermatol Venereol. 2013 Jun;27(6):699-705. doi: 10.1111/j.1468-3083.2012.04539.x. Epub 2012 Apr 4.
Muir-Torre syndrome (MTS) is an autosomal-dominant disorder characterized by the association of sebaceous tumors or keratoacanthomas with an early onset visceral cancer in the spectrum of Lynch syndrome.
A total of 20 sebaceous tumors including 18 sebaceous adenoma and two sebaceomas of six patients with MTS were analysed. Two main clinico-dermoscopic features were observed: (1) clinically pink to white papules/nodules with a central crater, dermoscopically characterized by radially arranged, elongated crown vessels surrounding opaque structureless yellow areas at times covered by blood crusts (n = 13) and (2), clinically pink to yellow papules/nodules without a central crater, dermoscopically exhibiting a few, loosely arranged yellow comedo-like globules and branching arborizing vessels (n = 7). Confocal microscopy was available in three sebaceous adenomas and revealed a good histopathologic correlation; sebaceous lobules were composed by clusters of ovoid cells with dark nuclei and bright, highly refractile glistening cytoplasm. They were delimited by a rim of epithelial cells, corresponding to basaloid cells.
A better characterization of clinical, dermoscopic and confocal microscopy features of sebaceous tumors may improve their recognition and consequently, aid to rise the suspect for MTS.
Muir-Torre 综合征（MTS）是一种常染色体显性遗传疾病，其特征是在 Lynch 综合征范围内，存在皮脂腺肿瘤或角化棘皮瘤与早期内脏癌的关联。
共分析了 6 例 MTS 患者的 20 个皮脂腺肿瘤，包括 18 个皮脂腺腺瘤和 2 个皮脂瘤。观察到两个主要的临床-皮肤镜特征：（1）临床上为粉红色至白色丘疹/结节，中央有凹陷，皮肤镜下表现为放射状排列的拉长的冠状血管，周围环绕着不透明的无结构黄色区域，有时覆盖着血痂（n = 13）；（2）临床上为粉红色至黄色丘疹/结节，中央无凹陷，皮肤镜下显示少量松散排列的黄色似粉刺样球和分支状树枝状血管（n = 7）。有 3 个皮脂腺腺瘤可进行共聚焦显微镜检查，显示出良好的组织病理学相关性；皮脂腺小叶由簇状卵圆形细胞组成，细胞核深染，细胞质明亮，高度折光。它们被一层上皮细胞围绕，对应于基底样细胞。
更好地描述皮脂腺肿瘤的临床、皮肤镜和共聚焦显微镜特征可能有助于提高对其的识别，从而提高对 MTS 的怀疑。
