Süss R, Megahed M, Zumdick M, Glover M, Ruzicka T, Lehmann P
Hautklinik, Heinrich-Heine-Universität, Düsseldorf.
Hautarzt. 1996 Jul;47(7):541-4. doi: 10.1007/s001050050468.
Purpura fulminans must be treated as an emergency in internal medicine and dermatology. Its characteristic features are the sudden development of progressively enlarging haemorrhagic skin necrosis, severe disseminated intravascular coagulation with consumption of anticoagulant factors, and signs of shock. Purpura fulminans can be classified into a neonatal form with inherited protein C deficiency and an acquired type for which multiple causes are known. Clinically it is characterized by massive ecchymosis with haemorrhagic blebs and acral necrosis. Histologically the lesions show widespread extravasation of erythrocytes and thrombosis of small vessels. Thrombocytopenia, decrease of coagulation factors, the presence of fibrinogen split products and fragmented erythrocytes in the blood smear help to confirm the diagnosis. The therapy includes fresh-frozen plasma, heparin, antibiotics and surgical debridement of necrotic areas. It is important to recognize the disease promptly because the mortality rate is about 30-40% and only quick intervention helps to save the life of the patient.
暴发性紫癜在内科和皮肤科都必须作为一种急症来治疗。其特征为进行性扩大的出血性皮肤坏死突然出现、严重的弥散性血管内凝血伴抗凝因子消耗以及休克体征。暴发性紫癜可分为伴有遗传性蛋白C缺乏的新生儿型和已知多种病因的获得型。临床上其特征为伴有出血性水疱的大片瘀斑和肢端坏死。组织学上，病变表现为红细胞广泛外渗和小血管血栓形成。血小板减少、凝血因子减少、血涂片中有纤维蛋白原裂解产物和破碎红细胞有助于确诊。治疗包括新鲜冷冻血浆、肝素、抗生素以及对坏死区域进行手术清创。及时识别该疾病很重要，因为死亡率约为30% - 40%，只有迅速干预才能挽救患者生命。
