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在落叶型天疱疮流行地区携带桥粒芯糖蛋白1抗体的健康受试者的超微结构皮肤改变:病例系列

Ultrastructural skin alterations of healthy subjects with -desmoglein 1 antibodies in endemic areas to pemphigus foliaceus: A case series.

作者信息

Ramos Willy, Rojas Nancy, Ortega-Loayza Alex G, Tello Mercedes, Jiménez Gerardo, Cuba-Cáceres Nicolás, Ronceros Gerardo, De La Cruz-Vargas Jhony A, Vera-Ponce Víctor Juan, Guerrero Nadia, Gutierrez Ericson L

机构信息

Instituto de Investigaciones en Ciencias Biomédicas (INICIB), Universidad Ricardo Palma, Lima, Peru.

Laboratorio de Microscopía Electrónica, Instituto de Patología, Universidad Nacional Mayor de San Marcos, Lima, Peru.

出版信息

J Transl Autoimmun. 2023 Jul 13;7:100208. doi: 10.1016/j.jtauto.2023.100208. eCollection 2023 Dec.

Abstract

BACKGROUND

Endemic pemphigus foliaceus and endemic pemphigus vulgaris are autoimmune dermatologic disorders endemic to the Peruvian Amazon.

OBJECTIVE

To determine the ultrastructural skin alterations of three healthy subjects with anti DSG-1 antibodies in areas endemic to pemphigus foliaceus and pemphigus vulgaris in the Peruvian Amazon.

PATIENTS AND METHODS

Case series carried out from data of three clinically healthy subjects positive to anti DSG-1 antibodies, from Peru. This study consists of a sub-analysis of data gathered in a previous study.

RESULTS

Ultrastructural results are presented from the skin biopsies of three clinically healthy patients positive to -desmoglein 1 (DSG-1) antibodies. High Resolution Optical Microscopy (HROM) showed the absence of acantholysis. Transmission Electron Microscopy (TEM) showed the widening of intercellular space between keratinocytes, the presence of vacuoles in intercellular space with granular material and cytoplasmic vacuolization, loss of desmosome structure, loss of normal distribution among tonofilaments and lateral separation among cells in the stratum basale.

CONCLUSION

According to our results, healthy subjects that present -desmoglein 1 antibodies can develop ultrastructural alterations that are visible through transmission electron microscopy but not through conventional optical microscopy.

摘要

背景

地方性落叶型天疱疮和地方性寻常型天疱疮是秘鲁亚马逊地区特有的自身免疫性皮肤病。

目的

确定秘鲁亚马逊地区落叶型天疱疮和寻常型天疱疮流行区域内三名抗桥粒芯糖蛋白-1(DSG-1)抗体阳性健康受试者的皮肤超微结构改变。

患者和方法

对来自秘鲁的三名抗DSG-1抗体阳性的临床健康受试者的数据进行病例系列研究。本研究包括对先前研究收集的数据进行的子分析。

结果

呈现了三名抗桥粒芯糖蛋白1(DSG-1)抗体阳性临床健康患者皮肤活检的超微结构结果。高分辨率光学显微镜(HROM)显示无棘层松解。透射电子显微镜(TEM)显示角质形成细胞间细胞间隙增宽,细胞间隙有含颗粒物质的空泡及细胞质空泡化,桥粒结构丧失,张力丝正常分布丧失,基底层细胞间侧向分离。

结论

根据我们的结果,抗桥粒芯糖蛋白1抗体阳性的健康受试者可出现通过透射电子显微镜可见但常规光学显微镜不可见的超微结构改变。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c291/10372304/26c53625c130/gr1.jpg

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