Andrews B S, Friou G J, Barr R J, Mirick G R, Berman M, Sandborg C, Ross P A
J Rheumatol. 1986 Apr;13(2):341-8.
Skin biopsies from the volar aspect of the forearm were studied in 26 patients with progressive systemic sclerosis (PSS) (16 diffuse, 10 CREST) and 4 controls using monoclonal antibodies against Langerhans' cells, T lymphocytes, macrophages, B lymphocytes, NK/K cells and HLA-DR antigen(s). Langerhans' cells were reduced or absent (anti-T6, anti-HLA-DR) in 19 of 20 clinically involved and in all 6 uninvolved PSS skin biopsies. Electron microscopic studies of 3 PSS patients indicated a reduction in the number of Langerhans' cells, with normal morphology of the remaining. HLA-DR antigen(s) on dermal endothelial cells were absent or reduced in 8 of 20 involved and 5 of 6 uninvolved PSS skin biopsies, but were present on the surface of dermal mononuclear cells presumably representing activated T lymphocytes. Increased numbers of dermal macrophages were found in 19% of PSS biopsies compared with controls. Absence of Langerhans' cells appears to represent the most widespread immunopathological feature of PSS. It is also associated with absent endothelial HLA DR surface antigens and activated T lymphocytes within the dermis.
对26例进行性系统性硬化症（PSS）患者（16例弥漫型，10例CREST型）以及4例对照者，使用抗朗格汉斯细胞、T淋巴细胞、巨噬细胞、B淋巴细胞、NK/K细胞和HLA - DR抗原的单克隆抗体，研究了取自前臂掌侧的皮肤活检样本。在20例临床受累的PSS皮肤活检样本中的19例以及所有6例未受累的PSS皮肤活检样本中，朗格汉斯细胞减少或缺失（抗T6、抗HLA - DR）。对3例PSS患者的电子显微镜研究表明，朗格汉斯细胞数量减少，其余细胞形态正常。在20例受累的PSS皮肤活检样本中的8例以及6例未受累的PSS皮肤活检样本中的5例中，真皮内皮细胞上的HLA - DR抗原缺失或减少，但在可能代表活化T淋巴细胞的真皮单核细胞表面存在。与对照相比，在19%的PSS活检样本中发现真皮巨噬细胞数量增加。朗格汉斯细胞的缺失似乎是PSS最普遍的免疫病理特征。它还与真皮内内皮HLA DR表面抗原的缺失以及活化的T淋巴细胞有关。
