eb病毒相关皮肤坏死性血管炎的女孩杂合因子V Leiden。

Cristina Guerriero, Gaia Moretta, Giulia Bersani, Piero Valentini, Antonio Gatto, Donato Rigante
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引用次数: 2

摘要

背景:坏死性血管增生的基本特征是血管壁中性粒细胞浸润和坏死,它们可以作为原发性过程或继发于潜在疾病。虽然Henoch-Schönlein紫癜(HSp)是儿童期更常见的原发性血管炎,但有时必须将其与其他由感染、药物、疫苗或免疫介导的疾病引起的继发性血管炎区分开来。主要观察:我们报告一例14岁的女孩皮肤坏死性血管炎,出现在急性爱泼斯坦-巴尔病毒感染过程中。体格检查显示高度疼痛的红紫色病变,网状边缘局限于脚后。布洛芬对疼痛无反应,需要给予他他多和普瑞巴林。患者也是杂合因子V Leiden,这可能有助于皮肤疼痛病变的发展。结论:据我们所知,这是首例记录的儿童坏死性血管炎与急性EBV感染相关的病例,发生在Leiden因子V杂合的女孩身上。该患者皮肤表现的严重程度可能受伴发因子V Leiden的影响,导致高凝性和血管闭塞性病变伴明显的剧烈疼痛,这一症状在其他儿童血管疾病中很少见。
本文章由计算机程序翻译,如有差异,请以英文原文为准。

Epstein-Barr virus-related cutaneous necrotizing vasculitis in a girl heterozygous for factor V Leiden.

Epstein-Barr virus-related cutaneous necrotizing vasculitis in a girl heterozygous for factor V Leiden.

Epstein-Barr virus-related cutaneous necrotizing vasculitis in a girl heterozygous for factor V Leiden.

Epstein-Barr virus-related cutaneous necrotizing vasculitis in a girl heterozygous for factor V Leiden.

Background: Necrotizing vasculitides are basically characterized by vessel wall neutrophil infiltration and necrosis and they can occur as a primary process or secondary to an underlying disease. Although Henoch-Schönlein purpura (HSp) is the more frequent primary vasculitis in childhood, sometimes it has to be distinguished from other secondary vasculitides induced by infections, drugs, vaccines, or immune-mediated disorders.

Main observations: We report a case of a 14-year-old girl with cutaneous necrotizing vasculitis, appearing in the course of acute Epstein-Barr virus infection. Physical examination revealed highly aching erythematous-purple lesions with reticular edges localized on the back of feet. Pain was non-responsive to ibuprofen and required administration of tapentadol and pregabalin. The patient was also heterozygous for factor V Leiden that might have contributed to the development of cutaneous painful lesions.

Conclusions: To our knowledge this is the first documented pediatric case of necrotizing vasculitis associated with acute EBV infection in a girl heterozygous for factor V Leiden. In this patient the severity of skin manifestations might have been influenced by the concomitant factor V Leiden, which gave rise to hypercoagulability and occlusive vasculopathy with markedly severe pain, a symptom rather infrequent in other childhood vasculitides.

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