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[急性发热性嗜中性皮病(斯威特综合征)。嗜中性粒细胞相关蛋白水解酶及其抑制剂的病例报告和免疫组织学研究]

[Acute febrile neutrophilic dermatosis (Sweet syndrome). Case report and immunohistologic study of neutrophil-associated proteolytic enzymes and their inhibitors].

作者信息

Metz R, Frosch P J, Schirmer U, Kramer M D

机构信息

Hautklinik, Ruprecht-Karls-Universität Heidelberg.

出版信息

Hautarzt. 1990 Sep;41(9):485-9.

PMID:2249945
Abstract

A 59-year-old female patient who developed fever, headache, arthralgia and painful, raised red cutaneous plaques located predominantly on the upper trunk is presented. On the basis of the clinical and histological findings acute febrile neutrophilic dermatosis (Sweet's syndrome) was diagnosed. In affected skin we applied immunohistochemical methods to examine the topographical organization of neutrophil-associated proteinase/proteinase inhibitor systems, i.e. the PMN-elastase/alpha 1-proteinase inhibitor and the cathepsin G/alpha 1-antichymotrypsin systems. Cathepsin G, PMN-elastase and alpha 1-antichymotrypsin were found in association with the polymorphonuclear infiltrating cells, whereas alpha 1-proteinase inhibitor was localized exclusively in the subepidermal oedema. In conclusion, our immunohistological findings support the view that free PMN-elastase is involved in the pathogenesis of cutaneous lesions in Sweet's syndrome.

摘要

本文报告了一名59岁女性患者,该患者出现发热、头痛、关节痛,主要位于上躯干的皮肤出现疼痛性、凸起的红色斑块。根据临床和组织学检查结果,诊断为急性发热性嗜中性皮病(Sweet综合征)。在受累皮肤中,我们采用免疫组化方法检查嗜中性粒细胞相关蛋白酶/蛋白酶抑制剂系统的拓扑结构,即PMN弹性蛋白酶/α1-蛋白酶抑制剂和组织蛋白酶G/α1-抗糜蛋白酶系统。发现组织蛋白酶G、PMN弹性蛋白酶和α1-抗糜蛋白酶与多形核浸润细胞相关,而α1-蛋白酶抑制剂仅定位于表皮下水肿中。总之,我们的免疫组织学研究结果支持以下观点:游离的PMN弹性蛋白酶参与了Sweet综合征皮肤病变的发病机制。

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