IgG autoantibodies to type VII collagen and an exclusive IgG3 reactivity to the laminin α3 chain in a patient with an autoimmune subepidermal blistering disease
IgG autoantibodies to type VII collagen and an exclusive IgG3 reactivity to the laminin α3 chain in a patient with an autoimmune subepidermal blistering disease
复制标题
DOI：
10.1016/j.jaad.2005.05.001
复制
发表时间：
2005-09-01
影响因子：
13.8
通讯作者：
Sitaru, C
中科院分区：
文献类型：
--
作者：
Baican, A;Hirako, Y;Sitaru, C
我们描述了一例皮肤广泛病变的患者，循环中同时存在针对III型胶原和层粘连蛋白5的抗体。虽然针对III型胶原的自身抗体属于IgG2、IgG3和IgG4亚类，但层粘连蛋白5仅被IgG3自身抗体靶向。有趣的是，尽管存在IgG3自身抗体，但患者的血清未能固定皮肤-表皮交界处的补体。此外，这些自身抗体不会招募和激活白细胞，也不会导致冷冻切开的皮肤中的真皮-表皮分离。我们报告了一例最罕见的自身免疫性真皮下水泡，对层粘连蛋白5有特异性的IgG3反应。
We describe a patient with widespread skin lesions and circulating IgG autoantibodies to both type VII collagen and laminin 5. Although autoantibodies to type VII collagen belonged to IgG2, IgG3, and IgG4 subclasses, laminin 5 was targeted exclusively by IgG3 autoantibodies. Interestingly, despite the presence of IgG3 autoantibodies, the patient's serum failed to fix complement to the dermoepidermal junction. In addition, these autoantibodies did not recruit and activate leukocytes or induce dermoepidermal separation in skin sectioned by cryostat. We report a most unusual case of an autoimmune subepidermal blistering with an exclusive IgG3 reactivity to laminin 5.