Tada J, Hashimoto K
Department of Dermatology, Wayne State University School of Medicine, Detroit, Michigan 48201, USA.
J Cutan Pathol. 1996 Dec;23(6):511-7. doi: 10.1111/j.1600-0560.1996.tb01443.x.
We noticed intracytoplasmic membranous, annular, or circular structures in the lesion of pemphigus foliaceus and studied these by regular transmission electron microscopy and immunoelectron microscopy. These curvicircular bodies were observed in the preacantholytic keratinocytes of the blister wall as well as in acantholytic cells in 6 out of 6 patients with pemphigus foliaceus. They were absent in samples from 3 patients with pemphigus vulgaris. These structures were about 60-70 nm wide and consisted of 4 electron-dense layers. They were continuous with intact desmosomal structures and gap junctions in the periphery of the keratinocytes. These curvicircular membranous bodies were well labeled with immunogold particles for desmoglein, plakoglobin, connexin 43, and IgG. In contrast to pemphigus vulgaris, splitting of desmosomes through dissolution of intercellular desmoglea was seldom observed in all 6 specimens of pemphigus foliaceus. These findings suggest that in pemphigus foliaceus 1) curvicircular bodies are derived from internalized desmosomes and gap junctions, and 2) cell-to-cell adhesions are weakened by this internalization and acantholysis is initiated, while in pemphigus vulgaris the dissolution of desmoglea is the initial event. It is suggested that in pemphigus foliaceus the binding of autoantibody induces internalization of many intact desmosomes and gap junctions rather than splitting them.
我们在落叶型天疱疮的皮损中发现了胞质内的膜性、环状或圆形结构,并通过常规透射电子显微镜和免疫电子显微镜对其进行了研究。在6例落叶型天疱疮患者的疱壁棘层松解前角质形成细胞以及棘层松解细胞中均观察到了这些弯曲线状体。3例寻常型天疱疮患者的样本中未发现这些结构。这些结构宽约60 - 70纳米,由4个电子致密层组成。它们与角质形成细胞周边完整的桥粒结构和缝隙连接相连。这些弯曲线状膜性小体用抗桥粒芯糖蛋白、桥粒斑珠蛋白、连接蛋白43和IgG的免疫金颗粒标记良好。与寻常型天疱疮不同,在所有6例落叶型天疱疮标本中很少观察到通过细胞间桥粒芯糖蛋白溶解导致的桥粒分裂。这些发现表明,在落叶型天疱疮中:1)弯曲线状体源自内化的桥粒和缝隙连接;2)这种内化削弱了细胞间黏附并引发棘层松解,而在寻常型天疱疮中,桥粒芯糖蛋白的溶解是初始事件。有人提出,在落叶型天疱疮中,自身抗体的结合诱导许多完整桥粒和缝隙连接的内化而非使其分裂。