Agras Pinar Isik, Ozdemir Handan, Baskin Esra, Ozbek Namik
Baskent University Department of Pediatric Nephrology, 6 Cadde No. 72/3, Bahcelievler, 06490 Ankara, Turkey.
Pediatr Dermatol. 2007 Jan-Feb;24(1):57-60. doi: 10.1111/j.1525-1470.2007.00335.x.
Symptomatic protein C deficiency is a rare condition. Vasculopathy associated with hypercoagulable state in protein C deficiency has also been reported rarely. We described a boy who was diagnosed as having homozygous protein C deficiency during the neonatal period, when he developed purpura fulminans. At 7 years of age, he developed recurrent, painful, nonscarring, purpuric skin lesions. Histopathologic skin findings were compatible with those of vasculopathy. The histopathologic characteristics of these vasculopathic lesions and the pathogenetic mechanisms of their association with protein C deficiency are discussed.
有症状的蛋白C缺乏症是一种罕见的病症。与蛋白C缺乏症中的高凝状态相关的血管病变也鲜有报道。我们描述了一名男孩，他在新生儿期出现暴发性紫癜时被诊断为纯合子蛋白C缺乏症。7岁时，他出现了反复发作的、疼痛的、无瘢痕的紫癜性皮肤病变。皮肤组织病理学检查结果与血管病变相符。本文讨论了这些血管病变的组织病理学特征及其与蛋白C缺乏症相关的发病机制。
