Caux F, Aractingi S, Scrobohaci M L, Fauvel-Lafève F, Arbeille B, Baccard M, Dubertret L
Clinique des Maladies Cutanées, Hôpital Saint-Louis, Paris.
Ann Dermatol Venereol. 1994;121(8):537-42.
Degos' disease is a rare dermatosis characterized by papular lesions with a porcelain-white central atrophy and histopathological aspect of wedge-shaped infarction necrosis and an endovasculitis in the dermis. Its pathogenesis is unknown but many abnormalities of haemostasis have been reported.
Platelets functions, coagulation and fibrinolysis were estimated in three patients with Degos' disease. For one patient, direct immunoelectron microscopy using an antibody to von Willebrand factor was performed on lesional skin.
In all the patients, prolonged euglobulin lysis time, increased plasminogen activator (PA) and plasminogen activator inhibitor (PAI) activities before and after a venous occlusion test were detected and indicated an inhibition of fibrinolysis. Electron microscopy demonstrated in one case an increased number of Weibel-Palade bodies and a raised staining of von Willebrand factor in endothelial cells. Tests for coagulation and circulating anticoagulant were normal. Results of platelets adhesion showed decrease of adhesion in one case and increased adhesion in another. Platelets aggregation studies were normal in two cases and showed hyperactive spontaneous and induced aggregation in one case.
We showed an inhibition of fibrinolysis in three patients with Degos' disease. These abnormalities could induce a prethrombotic state. The release of PA and PAI from the endothelial cells into the blood stream and the modifications observed with electron microscopy may signify a primary lesion of endothelial cell of still unknown origin.
迪戈斯病是一种罕见的皮肤病，其特征为丘疹性病变，中央有瓷白色萎缩，组织病理学表现为楔形梗死坏死以及真皮内血管炎。其发病机制尚不清楚，但已有许多止血异常的报道。
对三名迪戈斯病患者的血小板功能、凝血和纤溶进行了评估。对一名患者的皮损皮肤进行了使用抗血管性血友病因子抗体的直接免疫电子显微镜检查。
所有患者均检测到优球蛋白溶解时间延长，静脉闭塞试验前后纤溶酶原激活物（PA）和纤溶酶原激活物抑制剂（PAI）活性增加，提示纤溶受到抑制。电子显微镜检查在一例中显示魏贝尔-帕拉德小体数量增加，内皮细胞中血管性血友病因子染色增强。凝血和循环抗凝剂检测正常。血小板黏附试验结果显示一例黏附降低，另一例黏附增加。两例血小板聚集研究正常，一例显示自发和诱导聚集亢进。
我们发现三名迪戈斯病患者存在纤溶抑制。这些异常可能导致血栓前状态。PA和PAI从内皮细胞释放到血流中以及电子显微镜观察到的改变可能意味着内皮细胞的原发性病变，其起源尚不清楚。
