Haemophilia Centre, Yorkhill Children's Hospital, Glasgow, UK.
Arch Dis Child. 2011 Nov;96(11):1066-71. doi: 10.1136/adc.2010.199919. Epub 2011 Jan 12.
Purpura fulminans (PF) is a haematological emergency in which there is skin necrosis and disseminated intravascular coagulation. This may progress rapidly to multi-organ failure caused by thrombotic occlusion of small and medium-sized blood vessels. PF may complicate severe sepsis or may occur as an autoimmune response to otherwise benign childhood infections. PF may also be the presenting symptom of severe heritable deficiency of the natural anticoagulants protein C or protein S. Early recognition and treatment of PF is essential to reduce mortality and to prevent major long-term health sequelae. However, management strategies require accurate identification of the underlying cause. This review focuses on the clinical features, differential diagnosis and laboratory features of the range of PF disorders and includes expert consensus opinion about immediate and on-going management.
暴发性紫癜（PF）是一种血液学急症，其特征为皮肤坏死和弥散性血管内凝血。这种情况可能迅速进展为小血管和中等大小血管血栓闭塞引起的多器官衰竭。PF 可能是严重脓毒症的并发症，也可能是对其他良性儿童感染的自身免疫反应。PF 也可能是严重遗传性抗凝蛋白 C 或蛋白 S 缺乏症的首发症状。早期识别和治疗 PF 对于降低死亡率和预防重大长期健康后遗症至关重要。然而，管理策略需要准确识别潜在病因。本综述重点介绍了一系列 PF 疾病的临床特征、鉴别诊断和实验室特征，并包含了关于即刻和持续管理的专家共识意见。
