Introduction:Blue rubber bleb nevus syndrome (BRBNS) is an uncommon vascular anomaly characterized by multifocal cutaneous, visceral, and other soft tissue or solid organ venous malformations. We observed that BRBNS lesions express immunohistochemical markers of lymphatic differentiation.Methods:BRBNS histopathologic specimens assessed at our institution during the past 27 years were reviewed. Slides from 19 BRBNS lesions were selected from 14 patients (9 cutaneous, 9 gastrointestinal, and 1 hepatic). We recorded the involved anatomical compartments and presence/absence of thrombi or vascular smooth muscle. Immunohistochemical endothelial expression of PROX1 (nuclear) and D2-40 (membranous/cytoplasmic) was evaluated semi-quantitatively.Results:Endothelial PROX1 immunopositivity was noted in all specimens; the majority (89.5%) demonstrated staining in more than 10% of cells. D2-40 immunopositivity was present in one-third (33%) of cutaneous lesions and only 1 gastrointestinal lesion.Conclusion:Endothelial cells in BRBNS almost always express 1 or more immunohistochemical markers of lymphatic differentiation.

中文翻译：

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蓝橡胶泡痣综合征淋巴内皮标志物的免疫组织化学表达

简介：蓝色橡胶泡痣综合征（BRBNS）是一种罕见的血管异常，其特征是多灶性皮肤、内脏和其他软组织或实体器官静脉畸形。我们观察到 BRBNS 病变表达淋巴分化的免疫组织化学标志物。方法：回顾了过去 27 年在我们机构评估的 BRBNS 组织病理学标本。来自 14 名患者（9 名皮肤病灶、9 名胃肠病灶和 1 名肝脏病灶）的 19 个 BRBNS 病灶的切片。我们记录了所涉及的解剖区室以及血栓或血管平滑肌的存在/不存在。半定量评估 PROX1（核）和 D2-40（膜/细胞质）的免疫组织化学内皮表达。结果：所有标本中均显示内皮 PROX1 免疫阳性；大多数（89.5%）超过 10% 的细胞出现染色。三分之一(33%)的皮肤病灶中存在D2-40免疫阳性，仅1例胃肠道病灶呈D2-40免疫阳性。结论：BRBNS中的内皮细胞几乎总是表达1种或多种淋巴分化的免疫组化标志物。