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81名波兰族患者寻常型天疱疮脐部受累的临床意义:两个罕见部位皮损分布的比较分析

Clinical significance of umbilical region involvement in pemphigus vulgaris in a series of 81 ethnic Poles: a comparative analysis of the distribution of lesions in two infrequent locations.

作者信息

Jałowska Magdalena, Gornowicz-Porowska Justyna, Seraszek-Jaros Agnieszka, Bowszyc-Dmochowska Monika, Kaczmarek Elżbieta, Dmochowski Marian

机构信息

Autoimmune Blistering Dermatoses Section, Department of Dermatology, Poznan University of Medical Sciences, Poznan, Poland.

Department and Division of Practical Cosmetology and Skin Diseases Prophylaxis, Poznan University of Medical Sciences, Poznan, Poland.

出版信息

Postepy Dermatol Alergol. 2022 Apr;39(2):281-285. doi: 10.5114/ada.2021.102857. Epub 2021 Jan 20.

DOI:10.5114/ada.2021.102857
PMID:35645684
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC9131967/
Abstract

INTRODUCTION

Autoimmune bullous diseases are potentially life-threatening dermatoses which present with cutaneous and/or mucosal blisters, diagnosed on the basis of clinical manifestations, direct immunofluorescence of perilesional tissue, and serum testing for circulating autoantibodies. Sometimes, lesions in the navel can lead to the diagnosis of a bullous disease.

AIM

To assess the frequency of occurrence of pemphigus lesions located in the navel area and nail apparatus in pemphigus vulgaris (PV) in ethnic Poles.

MATERIAL AND METHODS

Eighty one patients (31 males and 50 females, mean age 59 years) with dermatoses of the PV group diagnosed in 2002-2020 were retrospectively analysed using their photographic files. Statistical analysis was performed using the difference test between two proportions to check the difference between the percentage of PV patients with navel area involvement and nail apparatus involvement.

RESULTS

There was no statistically significant difference between PV patients with nail apparatus involvement (12.3%) and navel area involvement (14.8%) ( = 0.4632). Only females had lesions in the navel area in our series of PV patients.

CONCLUSIONS

It is speculated that the causal relationship may exist between the female reproductive system and the pattern of expression of PV lesions around the umbilicus. The awareness that PV can infrequently affect the umbilical region and the nail apparatus should help in some cases to establish the diagnosis of PV. The periumbilical involvement can facilitate the performance of DIF in individuals with lesions in less accessible areas.

摘要

引言

自身免疫性大疱性疾病是潜在危及生命的皮肤病,表现为皮肤和/或黏膜水疱,根据临床表现、皮损周围组织的直接免疫荧光以及循环自身抗体的血清检测进行诊断。有时,脐部病变可有助于大疱性疾病的诊断。

目的

评估波兰族寻常型天疱疮(PV)患者脐部和甲装置出现天疱疮皮损的发生率。

材料与方法

回顾性分析2002年至2020年诊断为PV组皮肤病的81例患者(31例男性和50例女性,平均年龄59岁)的照片档案。采用两个比例的差异检验进行统计分析,以检验脐部受累和甲装置受累的PV患者百分比之间的差异。

结果

甲装置受累的PV患者(12.3%)和脐部受累的PV患者(14.8%)之间无统计学显著差异( = 0.4632)。在我们的PV患者系列中,只有女性在脐部有皮损。

结论

推测女性生殖系统与脐周PV皮损的表达模式之间可能存在因果关系。认识到PV可罕见地累及脐部区域和甲装置在某些情况下有助于PV的诊断。脐周受累可便于对皮损位于较难触及区域的个体进行直接免疫荧光检查。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c0e4/9131967/5b0a5ec28b1a/PDIA-39-43081-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c0e4/9131967/5b0a5ec28b1a/PDIA-39-43081-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c0e4/9131967/5b0a5ec28b1a/PDIA-39-43081-g001.jpg

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Front Immunol. 2019 Aug 20;10:1974. doi: 10.3389/fimmu.2019.01974. eCollection 2019.
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[Pemphigus vulgaris in pediatrics: A case report].[小儿寻常型天疱疮:一例报告]
关注问题:自身免疫性大疱性疾病的命名需要更新,寻常型天疱疮倾向于影响紧邻自然腔道的部位,将该疾病看似多样的临床表现统一起来。
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