Dyskeratotic cells in persistent pruritic skin lesions as a prognostic factor in adult-onset Still disease
Dyskeratotic cells in persistent pruritic skin lesions as a prognostic factor in adult-onset Still disease
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DOI：
10.1097/md.0000000000019051
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发表时间：
2020-02-01
期刊：
影响因子：
1.6
通讯作者：
Terada, Yoshio
中科院分区：
文献类型：
--
作者：
Maeda-Aoyama, Natsuki;Hamada-Ode, Kazu;Terada, Yoshio
成人发病Still病（AOSD）是一种全身性炎症性疾病，以高热、短暂皮疹、关节炎和高铁血症为特征。据报道，AOSD还与其他皮肤病变有关，包括持续性瘙痒性丘疹和斑块。本研究旨在评估日本AOSD患者角化异常皮肤病变的意义。我们回顾性评估了20名日本AOSD患者持续瘙痒性皮肤病变和短暂性皮疹的组织学，以及角化异常细胞、血清标志物和预后之间的关系，并将AOSD的组织学与皮肌炎（DM）、药疹和移植物抗宿主病（GVHD）的组织学进行了比较。结果显示，持续性搔痒病变的特征是分散的单个角化细胞，凋亡外观局限于表皮上层和角质层，无炎症浸润。与AOSD相反，DM、药疹和GVHD的组织学显示，表皮各层都有角化异常细胞，并有炎症浸润。伴有短暂皮疹的AOSD未见角化异常细胞。瘙痒性AOSD病变中角化异常细胞的ssDNA和末端脱氧核苷酸转移酶介导的dUTP缺口末端标记呈阳性，表明细胞凋亡。有角化异常细胞的AOSD患者血清IL-18明显高于无角化异常细胞的AOSD患者，通常需要更高剂量的糖皮质激素、免疫抑制剂和生物制剂。10例有角化异常细胞的AOSD患者中有2例死于噬血细胞性淋巴组织细胞增多症。总之，持续性瘙痒性AOSD皮肤病变的特征是角化异常细胞具有凋亡特征，累及表皮上层。这可能与IL-18升高有关。这种角化不良可能是一种不良预后指标。
Adult-onset Still disease (AOSD), a systemic inflammatory disorder, is characterized by high fever, evanescent rash, arthritis, and hyperferritinaemia. AOSD is also reported to be associated with other skin lesions, including persistent pruritic papules and plaques. This study aimed to assess the significance of dyskeratotic skin lesions in Japanese AOSD patients. We retrospectively assessed the histology of persistent pruritic skin lesions and evanescent rashes and the relationship between dyskeratotic cells, serum markers, and outcomes in 20 Japanese AOSD patients, comparing AOSD histology with that of dermatomyositis (DM), drug eruptions, and graft-versus-host disease (GVHD). As the results, Persistent pruritic lesions were characterized by scattered single keratinocytes with an apoptotic appearance confined to the upper layer of the epidermis and horny layer without inflammatory infiltrate. In contrast to AOSD, the histology of DM, drug eruption, and GVHD demonstrated dyskeratotic cells in all layers of the epidermis with inflammatory infiltrate. AOSD with evanescent rash showed no dyskeratotic cells. The dyskeratotic cells in pruritic AOSD lesions stained positive for ssDNA and terminal deoxynucleotidyl transferase-mediated dUTP nick end-labeling, indicating apoptosis. Serum IL-18 was significantly higher in AOSD patients with dyskeratotic cells than those without, and generally required higher doses of glucocorticoids, immunosuppressants, and biologic agents. Two of ten AOSD patients with dyskeratotic cells died from hemophagocytic lymphohistiocytosis. In conclusion, Persistent pruritic AOSD skin lesions are characterized by dyskeratotic cells with apoptotic features, involving the upper layers of the epidermis. There may be a link to elevated IL-18. This dyskeratosis may be a negative prognostic indicator.