Department of Dermatology, Seoul National University Boramae Hospital, Seoul, South Korea.
J Eur Acad Dermatol Venereol. 2010 Feb;24(2):163-7. doi: 10.1111/j.1468-3083.2009.03370.x. Epub 2009 Jul 14.
Eruptive pseudo-angiomatosis (EPA) is a rare, relatively newly described cutaneous disorder characterized by the sudden onset of several bright red, angioma-like papules surrounded by blanched halo. Its aetiology is unknown; however, viral infection or mosquito bites have been speculated as possible causes.
This study aims to determine the clinical and histopathological features of EPA, and whether it is associated with Epstein-Barr virus (EBV) infection.
We conducted a retrospective chart review of 25 EPA cases from 2006 to 2008. In order to determine latent EBV infection, EBV-encoded small RNA (EBER) in situ hybridization was performed in 18 subjects. To determine EPA's distinguishing histological characteristics, we compared the cases with 22 control cases of perivascular lymphocytic infiltration for haematoxylin and eosin, CD3, CD4, CD8, CD31 and c-kit staining patterns.
The patient sample's female-to-male ratio was 2.1 : 1, and the patients' age ranged from 5 to 79 years (average 46 years). The lesions appeared during the months of July to September in all but 3 patients. Skin biopsies demonstrated capillary ectasia with perivascular mononuclear cellular infiltrates in the upper dermis. Most patients were otherwise healthy, and routine laboratory results were all normal except in one patient who had diabetes. The skin lesions faded without any treatment in 1-2 weeks. Results of EBER in situ hybridization were all negative. The only histological distinguishing feature of EPA was the presence of intravascular neutrophils, which was found to be present in 19 of the 20 EPA cases (95%), in contrast to only 3 of the 22 control subjects (14%) (P < 0.0001).
The sudden onset of lesions during the summer months among our patients supports the 'paraviral eruption' concept of this probably underdiagnosed condition. The significant presence of intravascular neutrophils may be a diagnostic clue of EPA in South Korea.
突发性假性血管瘤病（EPA）是一种罕见的、相对较新描述的皮肤疾病，其特征是突然出现几个鲜红色的血管瘤样丘疹，周围有苍白的晕圈。其病因不明；然而，病毒感染或蚊子叮咬被认为是可能的原因。
本研究旨在确定 EPA 的临床和组织病理学特征，以及它是否与 Epstein-Barr 病毒（EBV）感染有关。
我们对 2006 年至 2008 年间的 25 例 EPA 病例进行了回顾性图表回顾。为了确定潜伏性 EBV 感染，我们对 18 例患者进行了 EBV 编码的小 RNA（EBER）原位杂交。为了确定 EPA 的鉴别性组织学特征，我们将这些病例与 22 例血管周围淋巴细胞浸润的对照病例进行了比较，比较了苏木精和伊红、CD3、CD4、CD8、CD31 和 c-kit 染色模式。
患者样本的男女比例为 2.1:1，年龄从 5 岁到 79 岁（平均 46 岁）。除 3 例患者外，所有患者的皮损均在 7 月至 9 月间出现。皮肤活检显示在上皮下真皮毛细血管扩张伴血管周围单核细胞浸润。大多数患者身体健康，常规实验室结果均正常，只有 1 例患者患有糖尿病。皮肤病变在 1-2 周内未经任何治疗消退。EBER 原位杂交结果均为阴性。EPA 的唯一组织学鉴别特征是血管内中性粒细胞的存在，在 20 例 EPA 病例中的 19 例（95%）中发现存在血管内中性粒细胞，而在 22 例对照病例中仅 3 例（14%）（P <0.0001）。
我们患者的皮损在夏季突然出现，支持这种可能被低估的疾病的“副病毒疹”概念。血管内中性粒细胞的大量存在可能是韩国 EPA 的一个诊断线索。
