Lee Joyce Siong-See, Kossard Steven, McGrath Michael A
Skin and Cancer Foundation Australia, Sydney, New South Wales, Australia.
Arch Dermatol. 2008 Sep;144(9):1175-82. doi: 10.1001/archderm.144.9.1175.
We encountered a distinct arteriolar histopathologic finding of lymphocytic vasculitis associated with a hyalinized fibrin ring in vessel lumina. Identical histologic findings have previously been described as macular arteritis.
We describe 5 women (mean age, 25 years; age range, 20-34 years) with persistent, slowly progressive, patchy and reticular hyperpigmentation associated with livedo racemosa affecting predominantly the lower limbs. In the biopsy samples, infiltration of muscular vessel wall by inflammatory cells, affecting small arteries of the dermosubcutaneous junction or superficial subcutis, was present. Of the infiltrate, 90% or more consisted of mononuclear cells, mainly lymphocytes with an admixture of histiocytes. Neutrophils and eosinophils were absent or scant. Inflammation was confined to the vicinity of the vessel and the immediate surrounding panniculus. A concentric fibrin ring involving the entire periphery of the lumina of affected vessels was present in all the patients. Laboratory investigation results revealed that 4 patients had antiphospholipid antibodies in their serum. One of these patients had a heterozygous mutation of the factor V Leiden gene. Conclusion We term this arteritis lymphocytic thrombophilic arteritis to reflect the histologic features that combine lymphocytic vascular inflammation with changes representing a thrombophilic endovasculitis.
我们遇到了一种独特的小动脉组织病理学表现，即淋巴细胞性血管炎，并伴有血管腔内透明变性的纤维蛋白环。相同的组织学表现先前被描述为黄斑动脉炎。
我们描述了5名女性（平均年龄25岁；年龄范围20 - 34岁），她们存在持续性、缓慢进展性、斑片状和网状色素沉着，伴有主要累及下肢的网状青斑。在活检样本中，炎症细胞浸润肌性血管壁，累及真皮皮下交界处或浅表皮下组织的小动脉。浸润细胞中90%或更多为单核细胞，主要是淋巴细胞，并混有组织细胞。中性粒细胞和嗜酸性粒细胞不存在或很少。炎症局限于血管附近及紧邻的皮下组织。所有患者的受累血管腔周边均出现同心性纤维蛋白环。实验室检查结果显示，4例患者血清中有抗磷脂抗体。其中1例患者存在因子V莱顿基因杂合突变。结论我们将这种动脉炎称为淋巴细胞性血栓形成性动脉炎，以反映其组织学特征，即淋巴细胞性血管炎症与代表血栓形成性血管内膜炎的改变相结合。
