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Reticular erythematous mucinosis: a review of patients' characteristics, associated conditions, therapy and outcome in 25 cases

机译:网状红斑性粘蛋白病:25例患者的特征,相关病症,治疗和预后

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摘要

BACKGROUND AND OBJECTIVES: Reticular erythematous mucinosis (REM) and lupus erythematosus tumidus (LET) share similarities. However, to our knowledge no study extensively compared the histological features of these two conditions. The aim of this study is to compare the histological and immunohistochemical features of REM and LET.\udMETHODS: We evaluated epidermal thickness, hyperkeratosis, dermo-epidermal junction changes, interstitial mucin deposition, vessel dilatation and pattern, type and density of the inflammatory infiltrate in 25 cases of REM and LET. Anti-CD3, anti-CD20, anti-CD68, anti-CD4, anti-CD8, anti-CD123, anti-CD2AP, anti-IgG and anti-C3 antibodies were tested in a subset of patients.\udRESULTS: Both diseases are characterized by perivascular dermal infiltrates of lymphocytes mainly CD4+ positive and increased dermal mucin. However, REM tended to show more scattered and more superficial lymphocytes with more superficial mucin and to have less frequent immunoglobulin and complement depositions along the dermo-epidermal junction. Plasmacytoid dendritic cells (PDCs) were less represented in REM, and were mainly found as single cells differently from LET.\udCONCLUSIONS: REM and LET present some differences in the infiltrate, including PDCs, the mucin deposition and the immunoreactant deposition at the dermo-epidermal junction that justify the distinction of the two diseases and suggest different pathogenetic mechanisms.
机译:背景与目的:网状红斑粘液病(REM)和红斑狼疮(LET)具有相似之处。然而,据我们所知,尚无广泛研究比较这两种情况的组织学特征。本研究的目的是比较REM和LET的组织学和免疫组化特征。\ ud方法:我们评估了表皮厚度,角化过度,真皮-表皮连接变化,间质粘蛋白沉积,血管扩张和模式,炎性浸润的类型和密度。在25例REM和LET中。在部分患者中测试了抗CD3,抗CD20,抗CD68,抗CD4,抗CD8,抗CD123,抗CD2AP,抗IgG和抗C3抗体。其特征是淋巴细胞的血管周围真皮浸润主要是CD4 +阳性和真皮粘蛋白增加。然而,REM倾向于显示更多的分散的和更多的表面淋巴细胞以及更多的表面粘蛋白,并且具有较少的免疫球蛋白和沿着真皮-表皮连接处的补体沉积。浆细胞样树突状细胞(PDC)在REM中的代表较少,主要发现为与LET不同的单细胞。\ ud结论:REM和LET在浸润中存在一些差异,包括PDC,粘蛋白沉积和真皮上免疫反应物沉积表皮交界处证明两种疾病的区别是合理的,并暗示了不同的致病机制。

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