Department of Internal Medicine (CS), Lehigh Valley Health Network, Allentown, Pennsylvania; and Pulmonary and Critical Care Medicine (DSL), Lehigh Valley Health Network, Allentown, Pennsylvania.
Am J Med Sci. 2013 Dec;346(6):514-6. doi: 10.1097/MAJ.0b013e31829e02d3.
Acute perturbations in the hemostatic balance of anticoagulation and procoagulation antecede the manifestation of purpura fulminans, a rare syndrome of intravascular thrombosis and hemorrhagic infarction of the skin. Hallmarks include small vessel thrombosis, tissue necrosis and disseminated intravascular thrombosis. The course may be rapidly fulminant resulting in multiorgan failure with thrombotic occlusion of the vasculature, leading to distal extremity ischemia and necrosis. Depletion of protein C (PC) has been emphasized in the pathogenesis. Early intravenous antibiotic administration and hemodynamic support are cornerstones in management. Herein, we report a case of pneumococcal sepsis-induced purpura fulminans limited to the skin in an asplenic adult patient without the development disseminated intravascular coagulation.
急性凝血和抗凝平衡的紊乱先于暴发性紫癜的表现，暴发性紫癜是一种罕见的皮肤血管内血栓形成和出血性梗死综合征。其特征包括小血管血栓形成、组织坏死和弥漫性血管内血栓形成。病程可能迅速恶化，导致多器官衰竭，血管血栓形成闭塞，导致远端肢体缺血和坏死。蛋白 C（PC）的耗竭在发病机制中受到重视。早期静脉内抗生素治疗和血流动力学支持是治疗的基石。本文报告了一例脾切除成人患者，在无弥漫性血管内凝血的情况下，由肺炎球菌败血症引起的仅限于皮肤的暴发性紫癜。
