Li Li, Zeng Yueping, Fang Kai, Xiao Yu, Jin Hongzhong, Ray Heather, Chen Jiang
Department of Dermatology, Peking Union Medical College Hospital, No. 1, Shuai Fu Yuan Street, Beijing, China 100720.
Am J Dermatopathol. 2012 Jun;34(4):e41-5. doi: 10.1097/DAD.0b013e31823583f5.
Pilomatricoma is a common benign neoplasm of the skin characterized by a solid cutaneous nodule of hair matrix origin. The anetodermal or lymphangiectatic variant of pilomatricoma is rare, and its bullous appearance is often associated with attenuated collagen and elastic fibrils and dilated lymphatic vessels in the overlying dermis. However, the tumors of anetodermic pilomatricoma have never been characterized at the molecular level, and the exact mechanism for their development is unknown. In this study, we evaluated histological and molecular features of a bullous pilomatricoma along with 5 control tumors and determined that tumors of both anetodermic and control pilomatricoma comprise similar molecular features, such as nuclear lymphoid enhancer binding factor 1 (LEF1) localization and the expression of keratins. In addition, we associated the development of the anetodermic pilomatricoma with mechanical trauma, scar tissue formation, and increased numbers of blood and lymphatic vessels. This study suggests that the development of the anetodermic form of pilomatricoma is unlikely to be associated with the intrinsic properties of the tumor but with the mechanical trauma that disrupts the dermal integrity and vascular microenvironment.
毛母质瘤是一种常见的皮肤良性肿瘤,其特征为起源于毛发基质的实性皮肤结节。毛母质瘤的无皮症或淋巴管扩张型变种较为罕见,其大疱样外观通常与上方真皮层中胶原蛋白和弹性纤维的减少以及淋巴管扩张有关。然而,无皮症型毛母质瘤的肿瘤在分子水平上从未被表征过,其确切的发生机制尚不清楚。在本研究中,我们评估了一例大疱性毛母质瘤以及5例对照肿瘤的组织学和分子特征,确定无皮症型和对照型毛母质瘤的肿瘤具有相似的分子特征,如核淋巴样增强子结合因子1(LEF1)定位和角蛋白表达。此外,我们将无皮症型毛母质瘤的发生与机械创伤、瘢痕组织形成以及血管和淋巴管数量增加联系起来。本研究表明,无皮症型毛母质瘤的发生不太可能与肿瘤的内在特性相关,而与破坏真皮完整性和血管微环境的机械创伤有关。