Department of Dermatology, The Second Xiangya Hospital, Central South University, Hunan Key Laboratory of Medical Epigenomics, Changsha, Hunan, PR China.
Department of Pathology, Tulane University School of Medicine, New Orleans, LA, USA.
Expert Rev Clin Immunol. 2019 Dec;15(12):1341-1350. doi: 10.1080/1744666X.2020.1685876. Epub 2019 Nov 4.
: Recently, atypical persistent skin eruptions (APSEs) have been documented as a new manifestation of adult-onset Still's disease (AOSD), with a unique pathological feature of necrotic keratinocytes in the upper third of the epidermis, but the mechanism has not been elucidated. The aim of this study was to explore the potential mechanism of the unique pathological phenomenon of APSEs.: Clinical and pathological data from 26 AOSD patients with APSEs and 6 with evanescent skin eruptions (ESEs) were reviewed. Fourteen APSE biopsies and 6 ESE biopsies were selected for multi-spectrum immunohistochemistry with 5 disease controls and 5 healthy controls.: The unique pathological manifestation was present in all APSE patients but was hardly found in ESE patients. There were more CD4 + T-cells infiltrated in the dermis of APSEs than in the dermis of ESEs. IL-1β and IFN-γ were specifically expressed in the upper third of the epidermis and were juxtaposed to the loci of the necrotic keratinocytes.: Our findings showed important cellular and molecular derangements related to the APSE-specific pathological phenomena and helped to understand the pathogenesis of dyskeratosis in the epidermis. The findings could also pave a way to explore an effective intervention to this potentially life-threatening disorder.
: 最近，非典型持续性皮肤疹（APSEs）被记录为成人Still 病（AOSD）的一种新表现，其独特的病理学特征是在上表皮的三分之一处存在坏死的角质形成细胞，但机制尚未阐明。本研究旨在探讨 APSEs 独特病理现象的潜在机制。: 回顾了 26 例 AOSD 伴 APSEs 和 6 例一过性皮肤疹（ESEs）患者的临床和病理资料。选择了 14 例 APSE 活检和 6 例 ESE 活检进行多光谱免疫组化，同时还选择了 5 例疾病对照和 5 例健康对照。: 所有 APSE 患者均存在独特的病理表现，而 ESE 患者几乎没有。APSEs 真皮中浸润的 CD4 + T 细胞比 ESEs 真皮中多。IL-1β 和 IFN-γ 特异性在上表皮的上三分之一表达，并与坏死角质形成细胞的位置相邻。: 我们的发现显示与 APSE 特异性病理现象相关的重要细胞和分子紊乱，有助于了解表皮角化不良的发病机制。这些发现还可能为探索这种潜在危及生命的疾病的有效干预措施铺平道路。
