Xie Yong, Zhang Xiaoyong, Inoue Yuji, Wakasugi Shouji, Makino Takamitsu, Ihn Hironobu
Department of Dermatology & Plastic and Reconstructive Surgery, Graduate School of Medical and Pharmaceutical Sciences, Kumamoto University, 1-1-1 Honjo, Kumamoto 860-8556, Japan.
Eur J Dermatol. 2008 Jan-Feb;18(1):50-4. doi: 10.1684/ejd.2008.0310. Epub 2007 Dec 18.
Scleroderma is a chronic autoimmune connective tissue disorder of unknown etiology that affects the microvasculature and loose connective tissue. Langerhans cells play an important role in the immune system of the skin. By immunohistochemistry we investigated the phenotypical characteristics of epidermal and dermal Langerhans cells and their spatial relationship with infiltrating lymphocytes in systemic scleroderma (SSc) and localized scleroderma. Skin samples were obtained from patients by 6 mm punch biopsy. Samples were stained with antibodies against CD1a and CD86. The number of cells stained with both antibodies in the dermal and epidermal infiltration was calculated. In contrast to normal skin, both types of scleroderma skin showed a marked increase in CD1a+ dermal Langerhans cells, whereas the number of CD1a+ cells in localized scleroderma was much higher than that in SSc (p < 0.05) either in the dermis or in the epidermis. The expression of CD86 was increased in the dermis of localized scleroderma compared with that in SSc or normal skin (p < 0.05). This study revealed that Langerhans cells may play an important role in the pathogenesis of scleroderma, especially in localized scleroderma. CD86 is predominantly expressed on dermal Langerhans cells in the lesional skin of localized scleroderma. Therefore, it might play an important role in the pathogenesis of localized scleroderma.
硬皮病是一种病因不明的慢性自身免疫性结缔组织疾病，会影响微血管和疏松结缔组织。朗格汉斯细胞在皮肤免疫系统中发挥重要作用。我们通过免疫组织化学研究了系统性硬皮病（SSc）和局限性硬皮病中表皮和真皮朗格汉斯细胞的表型特征及其与浸润淋巴细胞的空间关系。通过6毫米打孔活检从患者身上获取皮肤样本。样本用抗CD1a和抗CD86抗体染色。计算真皮和表皮浸润中两种抗体染色的细胞数量。与正常皮肤相比，两种类型的硬皮病皮肤中CD1a +真皮朗格汉斯细胞均显著增加，而局限性硬皮病中CD1a +细胞的数量在真皮或表皮中均远高于系统性硬皮病（p < 0.05）。与系统性硬皮病或正常皮肤相比，局限性硬皮病真皮中CD86的表达增加（p < 0.05）。这项研究表明，朗格汉斯细胞可能在硬皮病的发病机制中起重要作用，尤其是在局限性硬皮病中。CD86主要在局限性硬皮病皮损皮肤的真皮朗格汉斯细胞上表达。因此，它可能在局限性硬皮病的发病机制中起重要作用。
