Department of Neuroscience, Section of Molecular Medicine, University of Siena, Via Aldo Moro, 53100 Siena, Italy.
Hum Pathol. 2010 Mar;41(3):366-74. doi: 10.1016/j.humpath.2009.08.009. Epub 2009 Dec 11.
Vascular involvement is frequent in systemic sclerosis, but the role of the lymphatic vasculature is poorly known. Our aim was to evaluate lymphatic vessels in systemic sclerosis skin lesions. We studied skin forearm biopsies of 9 patients with systemic sclerosis and 7 age-matched controls. Lymphatic vessels were labeled with the monoclonal antibody D2-40 and blood vessels with a polyclonal antibody to von Willebrand Factor. All blood and lymphatic vessels present in each section were counted and total area, inner luminal area, and shape factors were measured. The number of blood and lymphatic vessels in papillary dermis was greater and their diameter lower than in reticular dermis both in systemic sclerosis and controls. In the reticular dermis, the number of lymphatic vessels was markedly reduced in systemic sclerosis (4.9 +/- 1.1 microm(-2) versus 8.9 +/- 1.2 microm(-2)P = .03), and a similar trend was observed in papillary dermis (8.4 +/- 3.7 microm(-2) versus 8.1 +/- 5.3 microm(-2)). Interestingly, the number of periglandular lymphatics in systemic sclerosis was not different from controls. The inner luminal area (possibly indicating compensatory dilation) of lymphatic vessels, particularly the periglandular ones, was greater in systemic sclerosis than in controls. No differences were observed in the number of blood vessels, but the percentage of blood vessel profiles (without lumen) was significantly less in systemic sclerosis both in papillary and in reticular dermis. In conclusion, our data show that skin lesions in systemic sclerosis are characterized by a selective rarefaction of lymphatic vasculature that spares periglandular vessels and that might have a pathogenic role in the evolution and in the clinical manifestations of the disease.
血管病变在系统性硬化症中很常见,但淋巴管的作用知之甚少。我们的目的是评估系统性硬化症皮肤病变中的淋巴管。我们研究了 9 例系统性硬化症患者和 7 名年龄匹配的对照者前臂皮肤活检。淋巴管用单克隆抗体 D2-40 标记,血管用多克隆抗体 von Willebrand 因子标记。对每个切片中的所有血液和淋巴管进行计数,并测量总面积、内腔面积和形状因子。在系统性硬化症和对照组中,真皮乳头层的血管和淋巴管数量较多,直径较小。在真皮网状层中,系统性硬化症患者的淋巴管数量明显减少(4.9 +/- 1.1 μm(-2)对 8.9 +/- 1.2 μm(-2),P =.03),在真皮乳头层也观察到类似的趋势(8.4 +/- 3.7 μm(-2)对 8.1 +/- 5.3 μm(-2))。有趣的是,系统性硬化症患者的腺周淋巴管数量与对照组无差异。淋巴管的内腔面积(可能表明代偿性扩张),特别是腺周淋巴管,在系统性硬化症中大于对照组。血管数量无差异,但在真皮乳头层和真皮网状层中,血管形态(无内腔)的百分比明显低于系统性硬化症。总之,我们的数据表明,系统性硬化症皮肤病变的特征是淋巴管的选择性稀疏,腺周血管不受影响,这可能在疾病的发生和临床表现中具有致病作用。