Auletta M J, Headington J T
Department of Dermatology, University of Michigan Medical School, Ann Arbor 48109-0602.
Arch Dermatol. 1988 Sep;124(9):1387-91. doi: 10.1001/archderm.124.9.1387.
Protein C, when activated, is a vitamin K-dependent serine protease that has anticoagulant and profibrinolytic activities. An increased risk of thrombotic disease is associated with deficiency of this enzyme. A neonate developing purpura fulminans with internal thrombotic complications and congenital absence of protein C is described. Family studies showed partial protein C deficiency in the subject's symptom-free parents and two of three siblings. Clinically and histopathologically similar lesions are also seen in two acquired conditions in which there is a thrombotic tendency: coumarin necrosis and purpura fulminans of childhood, both of which are probably the result of transient protein C deficiency. We conclude that purpura fulminans can represent a cutaneous marker of acquired or congenital protein C deficiency.
蛋白C被激活后是一种依赖维生素K的丝氨酸蛋白酶，具有抗凝和促纤溶活性。这种酶缺乏会增加血栓形成性疾病的风险。本文描述了一名患有暴发性紫癜并伴有内部血栓形成并发症且先天性缺乏蛋白C的新生儿。家族研究显示，该患儿无症状的父母以及三个兄弟姐妹中的两个存在部分蛋白C缺乏。在两种有血栓形成倾向的后天性疾病中，即香豆素坏死和儿童暴发性紫癜，也可见到临床和组织病理学上相似的病变，这两种疾病可能都是短暂性蛋白C缺乏的结果。我们得出结论，暴发性紫癜可能是获得性或先天性蛋白C缺乏的皮肤标志。
