Campanelli Alexandre, Kaya Gürkan, Ozsahin Ayse Hulya, La Scala Giorgio, Jacquier Cédric, Stauffer Mélanie, Boehlen Françoise, de Moerloose Philippe, Saurat Jean-Hilaire
Department of Dermatology, University Hospital of Geneva, Geneva, Switzerland.
Dermatology. 2004;208(3):262-4. doi: 10.1159/000077315.
Purpura fulminans is a thrombotic disease that can occur during infections, disseminated intravascular coagulation or in the context of an acquired or congenital protein C or S deficiency. Here we report the case of a 4-year-old child who developed, 5 days after a chickenpox infection, large painful ecchymotic, necrotizing and retiform plaques on the lower extremities. Laboratory analyses revealed very low protein S levels as well as anticardiolipin antibodies. Aggressive treatment by low-molecular-weight heparin, steroids, intravenous immunoglobulins and fresh frozen plasma was able to prevent the extension of the lesions and to correct the coagulation abnormalities. No lesions required skin grafting. As in our patient, an acquired protein S deficiency is probably responsible for most cases of purpura fulminans occurring after varicella, but the concomitant presence of antiphospholipid antibodies may also play a role.
暴发性紫癜是一种血栓形成性疾病,可发生于感染、弥散性血管内凝血期间,或在获得性或先天性蛋白C或S缺乏的情况下。在此,我们报告一例4岁儿童病例,该患儿在水痘感染5天后,下肢出现大片疼痛性瘀斑、坏死性和网状斑块。实验室分析显示蛋白S水平极低以及抗心磷脂抗体。通过低分子量肝素、类固醇、静脉注射免疫球蛋白和新鲜冷冻血浆进行积极治疗,成功预防了病变扩展并纠正了凝血异常。无需进行皮肤移植。与我们的患者情况相同,获得性蛋白S缺乏可能是水痘后发生的大多数暴发性紫癜病例的病因,但抗磷脂抗体的同时存在也可能起作用。