Alexander E, Provost T T
Arch Dermatol. 1987 Jun;123(6):801-10. doi: 10.1001/archderm.123.6.801.
We describe a group of patients with Sjögren's syndrome, who commonly present to dermatologists with cutaneous manifestations of vasculitis. Two specific clinically recognizable forms of cutaneous vasculitis predominate: palpable purpura of the lower extremities (Waldenström's benign hypergammaglobulinemic purpura) and urticarialike vasculitis. Two pathologic types of cutaneous vasculopathy are demonstrated, one leukocytoclastic and the other mononuclear. The leukocytoclastic vasculopathy is associated with high titers of Ro(SS-A) and La(SS-B) autoantibodies (detected by gel double-diffusion techniques) and general serohyperreactivity. In marked contrast, the mononuclear vasculopathy is associated with low titers of Ro(SS-A) and La(SS-B) autoantibodies (detected by enzyme-linked immunosorbent assay but not gel double-diffusion techniques) and general serohyporeactivity. Approximately 70% of patients with Sjögren's syndrome and cutaneous vasculitis have also developed peripheral and/or central nervous system disease. The pathogenesis of the nervous system disease is unknown, but preliminary data suggest a vasculopathy.
我们描述了一组干燥综合征患者，他们常因血管炎的皮肤表现而就诊于皮肤科医生。两种特定的、临床上可识别的皮肤血管炎形式较为常见：下肢可触及性紫癜（瓦尔登斯特伦良性高球蛋白血症性紫癜）和荨麻疹样血管炎。展示了两种皮肤血管病的病理类型，一种是白细胞破碎性的，另一种是单核细胞性的。白细胞破碎性血管病与高滴度的Ro(SS - A)和La(SS - B)自身抗体（通过凝胶双扩散技术检测）以及全身血清高反应性相关。与之形成显著对比的是，单核细胞性血管病与低滴度的Ro(SS - A)和La(SS - B)自身抗体（通过酶联免疫吸附测定法检测，但凝胶双扩散技术检测不到）以及全身血清低反应性相关。大约70%患有干燥综合征和皮肤血管炎的患者还出现了周围和/或中枢神经系统疾病。神经系统疾病的发病机制尚不清楚，但初步数据提示为血管病。
