Holtman J H, Neustadt D H, Klein J, Callen J P
School of Medicine, University of Louisville, KY.
J Rheumatol. 1990 Sep;17(9):1222-5.
The deficiency of second component of complement (C2d) is the most common hereditary complement deficiency. Patients with C2 deficiency are frequently associated with an auto-immune disease process, in particular, systemic lupus erythematosus (LE)-like syndrome and/or vasculitic syndrome or bacterial infections. C2d has been associated with the LE subset of subacute cutaneous LE (SCLE), the presence of anti-Ro (SSA) antibodies, and the human leukocyte antigen (HLA) types A10, B18, DR2. We describe the clinical, serologic and immunogenetic data in a patient with manifestations of Sjögren's syndrome who developed urticarial vasculitis and photosensitive annular SCLE which were effectively treated with oral dapsone. Our case illustrates the dynamic nature of LE.
补体第二成分（C2）缺乏是最常见的遗传性补体缺乏症。C2缺乏患者常与自身免疫性疾病过程相关，特别是系统性红斑狼疮（LE）样综合征和/或血管炎综合征或细菌感染。C2缺乏与亚急性皮肤型红斑狼疮（SCLE）的LE亚型、抗Ro（SSA）抗体的存在以及人类白细胞抗原（HLA）A10、B18、DR2类型相关。我们描述了一名患有干燥综合征的患者的临床、血清学和免疫遗传学数据，该患者出现了荨麻疹性血管炎和光敏性环形SCLE，口服氨苯砜治疗有效。我们的病例说明了红斑狼疮的动态性质。
