Int J Gynecol Pathol. 2022 Nov 1;41(6):636-641. doi: 10.1097/PGP.0000000000000829. Epub 2021 Sep 29.
Acquired elastotic hemangioma (AEH) is a rare variant of hemangioma that usually presents as an asymptomatic, solitary, slow-growing red plaque on a sun-exposed site of an adult. Ultraviolet radiation can contribute to the pathogenesis of this hemangioma. Lichen sclerosus (LS), a scarring disease, may present a prominent accumulation of elastic fibers in the reticular dermis reflecting a reparative process. Vulvar elastosis, a novel diagnostic entity with fibers similar to solar elastosis, is more common in women 45 yr and older and is related to aging and/or hormonal changes. We herein report for the first time a case of AEH-like change located in the vulva, a sun-protected area, associated with genital LS. An 81-yr-old woman presented with a painful vulvar lesion of 1-yr duration. Clinical examination revealed vulvar LS and 1 cm-flat, erythematous, well-defined plaque with increased consistency located on the left labium minus. Histopathology showed a non-neoplastic proliferation of WT1-positive, small vascular channels surrounded and intertwined by intense elastosis in the reticular dermis. Alpha-smooth muscle actin positive pericytes encircled the vascular channels. The lesion can be understood as a reparative process within an LS with the appearance of epidermal hyperplasia, proliferation of small vascular channels, and hyperplastic elastosis. There is a close link between epidermal hyperplasia and angiogenesis in the formation of this reparative lesion. Recognition of this lesion is crucial to avoid confusion with other significant processes especially Kaposi sarcoma and well-differentiated angiosarcoma.
获得性弹性纤维血管瘤(AEH)是一种罕见的血管瘤变体,通常表现为成人暴露于阳光部位的无症状、单发、生长缓慢的红色斑块。紫外线辐射可能促成这种血管瘤的发病机制。硬皮病,一种瘢痕性疾病,网状真皮中可能会出现明显的弹性纤维堆积,反映出修复过程。外阴弹性纤维病是一种具有与日光性弹性纤维相似纤维的新诊断实体,在 45 岁及以上的女性中更为常见,与衰老和/或激素变化有关。我们在此首次报告一例位于外阴(受阳光保护的区域)的 AEH 样改变,与生殖器硬皮病有关。一名 81 岁女性因外阴疼痛性病变就诊,病程 1 年。临床检查显示外阴硬皮病和 1cm 扁平、红斑、界限清楚的斑块,质地稍硬,位于左小阴唇。组织病理学显示在网状真皮中,WT1 阳性的小血管通道呈非肿瘤性增生,周围和交织着强烈的弹性纤维。α-平滑肌肌动蛋白阳性的周细胞环绕着血管通道。该病变可被理解为硬皮病中的修复过程,表现为表皮增生、小血管通道增殖和增生性弹性纤维病。在这种修复性病变的形成中,表皮增生和血管生成之间存在密切联系。识别这种病变对于避免与其他重要过程(特别是卡波西肉瘤和分化良好的血管肉瘤)混淆至关重要。