Kim Min-Woo, Park Jung Tae, Kim Jung Ho, Koh Seong-Joon, Yoon Hyun-Sun, Cho Soyun, Park Hyun-Sun
Department of Dermatology, SMG-SNU Boramae Medical Center, Seoul, Korea.
Department of Pathology, SMG-SNU Boramae Medical Center, Seoul, Korea.
Ann Dermatol. 2017 Jun;29(3):268-275. doi: 10.5021/ad.2017.29.3.268. Epub 2017 May 11.
Periostin is a novel matricellular protein expressed in many tissues, including bone, periodontal ligament, and skin. Although its expression is prominent in various fibrotic conditions, studies of periostin in localized scleroderma are rare.
To investigate the expression of periostin and other molecules in localized scleroderma.
A retrospective study of 14 patients with confirmed mature stage localized scleroderma was undertaken. Fourteen age-matched and biopsy site-matched subjects with normal skin were included as controls. Collagen fiber deposition, periostin, procollagen, transforming growth factor-β, and matrix metalloproteinase (MMP)-1 expression were assessed and compared between the two groups. Co-localization of α-smooth muscle actin and periostin was evaluated using confocal microscopy.
Periostin was predominantly expressed along the dermo-epidermal junction in the controls. Conversely, patients with localized scleroderma demonstrated increased collagen fiber deposition and periostin expression that was more widely distributed along the entire dermis. MMP-1 staining showed increased expression in the epidermis and dermis of patients compared to scanty expression in the controls. A semi-quantitative evaluation showed a higher proportion of excessive collagen bundle deposition (57.1% vs. 7.1%, =0.013), diffuse periostin positivity (42.9% vs. 0%, =0.016), and moderate MMP-1 positivity (71.4% vs. 7.1%, =0.001) in patients than in the controls.
Compared to the controls, patients with localized scleroderma had enhanced periostin expression corresponding to increased collagen fiber deposition and unexpected overexpression of MMP-1. The results of this human study may implicate the pathogenesis of localized scleroderma.
骨膜蛋白是一种在包括骨骼、牙周韧带和皮肤在内的许多组织中表达的新型基质细胞蛋白。尽管其在各种纤维化疾病中表达显著，但关于骨膜蛋白在局限性硬皮病中的研究却很少。
研究骨膜蛋白及其他分子在局限性硬皮病中的表达情况。
对14例确诊为成熟阶段局限性硬皮病的患者进行回顾性研究。选取14例年龄匹配、活检部位匹配且皮肤正常的受试者作为对照。评估并比较两组之间的胶原纤维沉积、骨膜蛋白、前胶原、转化生长因子-β和基质金属蛋白酶（MMP）-1的表达情况。使用共聚焦显微镜评估α-平滑肌肌动蛋白和骨膜蛋白的共定位情况。
在对照组中，骨膜蛋白主要沿真皮-表皮交界处表达。相反，局限性硬皮病患者表现出胶原纤维沉积增加，且骨膜蛋白表达增加，其在整个真皮中分布更广泛。与对照组中少量表达相比，MMP-1染色显示患者的表皮和真皮中表达增加。半定量评估显示，患者中过度胶原束沉积比例更高（57.1%对7.1%，P=0.013）、弥漫性骨膜蛋白阳性比例更高（42.9%对0%，P=0.016）、MMP-1中度阳性比例更高（71.4%对7.1%，P=0.001）。
与对照组相比，局限性硬皮病患者骨膜蛋白表达增强，这与胶原纤维沉积增加以及MMP-1意外过表达相对应。这项人体研究结果可能与局限性硬皮病的发病机制有关。
