NEONATAL PURPURA FULMINANS DUE TO HOMOZYGOUS PROTEIN-C OR PROTEIN-S DEFICIENCIES
NEONATAL PURPURA FULMINANS DUE TO HOMOZYGOUS PROTEIN-C OR PROTEIN-S DEFICIENCIES
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DOI：
10.1055/s-2007-1002683
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发表时间：
1990-10-01
影响因子：
5.7
通讯作者：
NEUMANN, A
中科院分区：
文献类型：
--
作者：
MARLAR, RA;NEUMANN, A
新生儿暴发性紫癜，通常在出生后的头几天发作，是一种致命的疾病。迄今为止的证据表明，这是由于蛋白C或蛋白S完全缺乏所致1,2纯合蛋白C或蛋白S缺乏的新生儿首先在皮肤小血管中发生弥散性血管内凝血（DIC）和纤维蛋白或血小板血栓，随后发生真皮和皮下组织坏死和出血。1,3 -6如果不治疗，凝血功能障碍和皮肤病变会恶化，最终导致死亡。1暴发性紫癜是一种迅速扩散的皮肤病变，其特征是真皮层和真皮下小血管血栓形成，皮肤出血，出血区域形成大疱，其他部位无出血，无大血管血栓形成，死亡迅速。“暴发性紫癜”一词于1887年首次被描述，与良性感染后出现的一种获得性疾病有关。这些出血性皮肤病变出现在轻度或良性感染（水痘或链球菌）后，为皮肤形成紫癜性病变做了“准备”。7-9凝血异常与DIC一致，皮肤小静脉和毛细血管内广泛血栓形成是一致的病理表现。这些暴发性病变坏死，需要进行大清创或截肢。暴发性紫癜的发病和传播机制尚不完全清楚。10
Neonatal purpura fulminans, usually with onset during the first few days after birth, is a fatal disorder. Evidence to date shows it to be due to a complete deficiency of either protein C or protein S. 1, 2 The newborn with homozygous protein C or protein S deficiency first develops disseminated intravascular coagulation (DIC) and fibrin or platelet thrombi in the small vessels of the skin, followed by necrosis and hemorrhage into the dermis and subcutaneous tissues. 1, 3-6 If untreated, the coagulopathy and the skin lesions worsen and finally result in death. 1Purpura fulminans is a rapidly spreading lesion of the skin, characterized by thrombosis of the small vessels in the dermis and subdermis, bleeding into the skin, bullae formation in the hemorrhagic areas, absence of bleeding at other sites and no large vessel thrombosis, and rapid death. 7 The term" purpura fulminans" was first described in 1887 and was associated with an acquired condition manifesting after a benign infection. 7-9 These hemorrhagic skin lesions appear after a mild or benign infection (varicella or streptococcus), which" prepares" the skin for the generation of the purpuric lesion. 7-9 Coagulation abnormalities are compatible with DIC, and extensive thrombosis within the venules and capillaries of the skin are consistent pathologic findings. 7-9 These fulminating lesions become necrotic, necessitating major debridement or amputation. The mechanism of purpura fulminans initiation and propagation is not fully understood. 10