FIBRINOLYTIC ABNORMALITIES IN 3 PATIENTS WITH MALIGNANT ATROPHIC PAPULOSIS

Introduction. Degos' disease is a rare dermatosis characterized by pap ular lesions with a porclain-white central atrophy and histopathologic al aspect of wedge-shaped infarction necrosis and an endovasculitis in the dermis. Its pathogenesis is unknown but many abnormalities of hae mostasis have been reported. Patients and methods. Platelets functions , coagulation and fibrinolysis were estimated in three patients with D egos' disease. For one patient, direct immunoelectron microscopy using an antibody to von Willebrand factor was performed on lesional skin. Results. In all the patients, prolonged euglobulin lysis time, increas ed plasminogen activator (PA) and plasminogen activator inhibitor (PAI ) activities before and after a venous occlusion test were detected an d indicated an inhibition of fibrinolysis. Electron microscopy demonst rated in one case an increased number of Weibel-Palade bodies and a ra ised staining of von Willebrand factor in endothelial cells. Tests for coagulation and circulating anticoagulant were normal. Results of pla telets 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 on e case. Conclusion. We showed an inhibition of fibrinolysis in three p atients with Degos' disease. These abnormalities could induce a prethr ombotic state. The release of PA and PAI from the endothelial cells in to the blood stream and the modifications observed with electron micro scopy may signify a primary lesion of endothelial cell of still unknow n origin.