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Pathogenesis of pemphigus

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Author:
No author available
Journal Title:
International Journal of Dermatology and Venereology
Issue:
5
DOI:
10.3760/cma.j.issn.1673-4173.2017.05.013
Key Word:
天疱疮;桥粒;桥粒芯糖蛋白质类;基质金属蛋白酶类;HSP27热休克蛋白质类;Pemphigus;Desmosomes;Desmogleins;Matrix metalloproteinases;HSP27 heat-shock proteins

Abstract: Pemphigus is a chronic recurrent intraepidermal blistering skin disease caused by autoimmune response-induced epidermal acantholysis.It is characterized clinically by flaccid,thin-walled bullae arising on the erythematous skin,erosions and positive Nikolsky's sign.According to clinical manifestations,pemphigus can be classified into pemphigus vulgaris (PV),pemphigus vegetans (PVe),pemphigus foliaceus (PF) and pemphigus erythematous (PE).The main pathogenesis of pemphigus is the presence of autoantibodies against keratinocyte desmogleins (Dsgs) in patients,but exact mechanisms leading to acantholysis are still unclear.With the development of proteomics,immunology,molecular biological techniques and further insights into the pathogenesis of pemphigus in the past decades,it has been shown that besides anti-Dsg auto antibodies,non-Dsg antibodies are also involved in the formation of acantholvsis,which provids new potential therapeutic targets for pemphigus.

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