Departments of, Dermatology, Taichung Veterans General Hospital, Taichung, Taiwan.
Department of, Pathology, Taichung Veterans General Hospital, Taichung, Taiwan.
Australas J Dermatol. 2021 Feb;62(1):e102-e106. doi: 10.1111/ajd.13451. Epub 2020 Sep 25.
Recurrent cutaneous necrotising eosinophilic vasculitis (RCNEV) is a rare disease that was first described in 1994. We report a case of RCNEV treated with corticosteroid, and 18 cases that we identified in the literature. Our review of the literature shows that RCNEV was frequently identified in middle-aged females from Asia and usually presents as erythematous to purpuric papuloplaques, angio-oedema on the extremities, as well as peripheral eosinophilia. Histopathologically, RCNEV is characterised by exclusively eosinophilic infiltration around the vascular plexus, the absence of leukocytoclasis and fibrinoid degeneration of vascular walls. Although, RCNEV responds to corticosteroid treatment, relapses have occurred during dose tapering. We also discuss the mechanisms of vascular destruction, the differential diagnosis and steroid-sparing therapies for RCNEV.
复发性皮肤坏死性嗜酸性血管炎（RCNEV）是一种罕见的疾病，于 1994 年首次描述。我们报告了一例接受皮质类固醇治疗的 RCNEV 病例，并对文献中的 18 例病例进行了回顾。我们对文献的回顾表明，RCNEV 常见于来自亚洲的中年女性，通常表现为红斑至瘀斑性丘疹、四肢血管性水肿，以及外周血嗜酸性粒细胞增多。组织病理学上，RCNEV 的特征是仅在血管丛周围有嗜酸性粒细胞浸润，没有白细胞碎裂和血管壁纤维蛋白样变性。尽管 RCNEV 对皮质类固醇治疗有反应，但在剂量逐渐减少时会复发。我们还讨论了血管破坏的机制、RCNEV 的鉴别诊断和类固醇节省疗法。
