Kaneko F, Tsukinaga I, Ando M, Ohkawara A, Oguchi H, Oikawa K, Nagai M
Department of Dermatology, Hokkaido University School of Medicine, Japan.
Dermatologica. 1989;178(3):156-63. doi: 10.1159/000248416.
Activities of plasminogen activators (PA) and their inhibitors were studied in the bullous lesions of 2 siblings with recessive dystrophic epidermolysis bullosa (RDEB). The hematological findings of the patients revealed hyperfibrinogenemia, hyper-gamma-globulinemia and marked thrombocytosis. The immunofluorescent studies showed strong deposits of plasminogen, fibrin-degenerative products and alpha 1-antitrypsin around the blister lesions. On the other hand, alpha 2-macroglobulin (alpha 2-M) was sparsely deposited. The levels of alpha 2-M in the blister fluid of the patients were also decreased in comparison with those from patients with other bullous dermatoses. The topical application of antibiotic ointment with strong PA inhibitor was clinically effective when applied to the blister and eroded lesions of the patients. These findings suggest that the increased activity of PA may play an important role in the development of blister formation in patients with RDEB.
对2例患有隐性营养不良性大疱性表皮松解症（RDEB）的同胞兄妹的大疱性皮损进行了纤溶酶原激活剂（PA）及其抑制剂活性的研究。患者的血液学检查结果显示有高纤维蛋白原血症、高γ球蛋白血症和明显的血小板增多症。免疫荧光研究显示，在水疱皮损周围有强烈的纤溶酶原、纤维蛋白降解产物和α1抗胰蛋白酶沉积。另一方面，α2巨球蛋白（α2-M）沉积稀疏。与其他大疱性皮肤病患者相比，这些患者水疱液中的α2-M水平也有所降低。当将具有强效PA抑制剂的抗生素软膏局部应用于患者的水疱和糜烂皮损时，临床效果显著。这些发现表明，PA活性增加可能在RDEB患者水疱形成过程中起重要作用。
