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落叶型天疱疮的诊断:临床、组织学及免疫学标准的回顾性分析

Diagnosing Pemphigus foliaceus: a retrospective analysis of clinical, histological and immunological criteria.

作者信息

Delmonte S, Kanitakis J, Cozzani E, Parodi A, Rebora A

机构信息

Section of Dermatology, DiSEM, Genoa, Italy.

出版信息

Dermatology. 2001;203(4):289-93. doi: 10.1159/000051774.

Abstract

BACKGROUND

Clinical, histological and immunological criteria distinguish pemphigus foliaceus (PF) from pemphigus vulgaris (PV), but whether and how often they are concordant in the same patient is unknown.

METHODS

Seven clinical records were selected from two hospital settings for having a diagnosis of PF and the initial serum and histopathological specimens still available. Controls were 8 PV records selected in the same way. Histopathological slides were re-evaluated. Stored sera were studied by indirect immunofluorescence (IIF), Western blot and ELISA.

RESULTS

Acantholysis was superficial in all PF patients and deep in all PV patients. Mucosal lesions were not exclusive of PV. IIF was positive in 43% of PF patients. Western blot revealed desmoglein 1 in 86% of PF patients and in 25% of PV. ELISA revealed anti-desmoglein-1 antibodies in up to 71% of PF and in 62% of PV patients, in 1 failing to detect anti-desmoglein-3 antibodies.

CONCLUSIONS

Histopathology remains the most reliable criterion for diagnosing PF. Western blot and ELISA, especially in combination, may be only of confirmatory value.

摘要

背景

临床、组织学和免疫学标准可区分落叶型天疱疮(PF)和寻常型天疱疮(PV),但它们在同一患者中是否一致以及一致的频率尚不清楚。

方法

从两家医院选取7例诊断为PF且仍有初始血清和组织病理学标本的临床记录。对照组为以同样方式选取的8例PV记录。对组织病理学切片进行重新评估。通过间接免疫荧光(IIF)、蛋白质印迹法和酶联免疫吸附测定(ELISA)研究储存的血清。

结果

所有PF患者的棘层松解均为浅表性,所有PV患者的棘层松解均为深部。黏膜损害并非PV所特有。43%的PF患者IIF呈阳性。蛋白质印迹法显示,86%的PF患者和25%的PV患者存在桥粒芯糖蛋白1。ELISA显示,高达71%的PF患者和62%的PV患者存在抗桥粒芯糖蛋白-1抗体,1例未检测到抗桥粒芯糖蛋白-3抗体。

结论

组织病理学仍然是诊断PF最可靠的标准。蛋白质印迹法和ELISA,尤其是联合使用时,可能仅具有确诊价值。

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