Wilk M, Kaiser H W, Steen K H, Kreysel H W
Hautklinik und Poliklinik, Rheinischen Friedrich-Wilhelms-Universität, Bonn.
Hautarzt. 1995 Jun;46(6):413-6. doi: 10.1007/s001050050275.
The clinical and histological diagnosis of sclerotic fibroma is important because of its potential association with Cowden's syndrome. Despite its distinctive histological appearance the lesion is often mis-diagnosed. We therefore present five of our own cases (2F, 3M) in which the tumor was located on the head (n = 2), arm (n = 2) and leg (n = 1), respectively. Clinically, the lesions were white to flesh-colored firm nodules ranging in size from 0.5 to 1.2 cm. None of our patients revealed any clinical evidence of Cowden's disease. Simple surgical excision seems to be curative. Histologically, they were well-circumscribed but not encapsulated dermal nodules composed of stori-form-arranged sclerosing collagen bundles and vimentin-positive fibroblastlike cells interspersed in three cases by a number of alpha-smooth-muscle actin-positive myofibroblasts. Approximately 50% of cells (dermal dendritic cells (DD)) also reacted for factor XIIIa evenly scattered throughout the lesion in contrast to the very few (< 5%) CD34+ DD found predominantly at the lower border, thus possibly reflecting the distribution of these cells in normal skin. Sclerotic fibroma expands the spectrum of fibrous lesions that may express alpha-smooth-muscle actin.
硬化性纤维瘤的临床和组织学诊断很重要，因为它可能与考登综合征相关。尽管其组织学表现独特，但该病变常被误诊。因此，我们展示了我们自己的5例病例（2例女性，3例男性），肿瘤分别位于头部（n = 2）、手臂（n = 2）和腿部（n = 1）。临床上，病变为白色至肉色的坚实结节，大小从0.5厘米至1.2厘米不等。我们的患者均未显示考登病的任何临床证据。单纯手术切除似乎可治愈。组织学上，它们是边界清楚但无包膜的真皮结节，由 storiform 排列的硬化胶原束和波形蛋白阳性的成纤维细胞样细胞组成，三例中有一些α-平滑肌肌动蛋白阳性的肌成纤维细胞散在分布。大约50%的细胞（真皮树突状细胞（DD））也对因子 XIIIa 有反应，均匀分布于整个病变中，与之形成对比的是，极少（<5%）的 CD34+ DD 主要位于下缘，这可能反映了这些细胞在正常皮肤中的分布情况。硬化性纤维瘤扩展了可能表达α-平滑肌肌动蛋白的纤维性病变谱。
