Baican Adrian, Hirako Yoshiaki, Lazarova Zelmira, Yancey Kim B, Zillikens Detlef, Sitaru Cassian
Department of Dermatology, University of Medicine and Pharmacy Cluj-Napoca, Cluj-Napoca, Romania.
J Am Acad Dermatol. 2005 Sep;53(3):517-22. doi: 10.1016/j.jaad.2005.05.001.
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.
我们描述了一名患有广泛皮肤病变且循环中存在针对VII型胶原蛋白和层粘连蛋白5的IgG自身抗体的患者。尽管针对VII型胶原蛋白的自身抗体属于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，但患者血清未能使真皮表皮交界处的补体固定。此外，这些自身抗体不会募集和激活白细胞，也不会在低温恒温器切片的皮肤中诱导真皮表皮分离。我们报告了一例极为罕见的自身免疫性表皮下大疱病病例，其对层粘连蛋白5具有独特的IgG3反应性。
