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自身免疫性水疱性疾病的诊断:一家三级转诊中心的经验

Diagnostics of autoimmune blistering disorders: an experience of a single tertiary referral centre.

作者信息

Adaszewska Alicja, Woźniak Katarzyna, Kalińska-Bienias Agnieszka, Smolarczyk Katarzyna, Kowalewski Cezary

机构信息

Department of Dermatology and Immunodermatology, Medical University of Warsaw, Warsaw, Poland.

Department of Dermatology and Venereology, Medical University of Warsaw, Warsaw, Poland.

出版信息

Postepy Dermatol Alergol. 2022 Jun;39(3):446-453. doi: 10.5114/ada.2020.99972. Epub 2020 Nov 4.

Abstract

Autoimmune blistering disorders (AIBD) include a heterogeneous group of diseases characterized by the presence of autoantibodies against the structural antigens of the skin and mucous membranes. The gold standard of AIBD diagnostics is the detection of in vivo bound IgG/IgA and/or complement component 3 in the direct immunofluorescence of a perilesional biopsy. Various immunological techniques such as indirect immunofluorescence of different tissue substrates including monkey oesophagus, salt split skin, recombinant proteins of epidermis and basement membrane zone as well as ELISA systems and immunoblotting are used to characterize target antigens. Proper and early diagnosis is crucial for both treatment and prognosis since some AIBD may be associated with a malignant neoplasm.

摘要

自身免疫性水疱病(AIBD)包括一组异质性疾病,其特征是存在针对皮肤和黏膜结构抗原的自身抗体。AIBD诊断的金标准是在病损周围活检的直接免疫荧光检查中检测体内结合的IgG/IgA和/或补体成分3。各种免疫技术,如对包括猴食管、盐裂皮肤、表皮和基底膜区重组蛋白在内的不同组织底物进行间接免疫荧光检查,以及酶联免疫吸附测定(ELISA)系统和免疫印迹法,用于鉴定靶抗原。由于某些AIBD可能与恶性肿瘤相关,因此正确和早期诊断对治疗和预后都至关重要。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1739/9326910/4d09a1d3c148/PDIA-39-42087-g001.jpg

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