Pemphigus and Pemphigoid: From Disease Mechanisms to Druggable Pathways

被引：32

作者：

Ellebrecht, Christoph T. ^{[1
]}

Maseda, Damian ^{[1
]}

Payne, Aimee S. ^{[1
]}

机构：

[1] Univ Penn, Perelman Sch Med, Dept Dermatol, 421 Curie Blvd, Philadelphia, PA 19104 USA

来源：

JOURNAL OF INVESTIGATIVE DERMATOLOGY | 2022年 / 142卷 / 3 PT B期

关键词：

LINKED-IMMUNOSORBENT-ASSAY; DESMOGLEIN; MONOCLONAL-ANTIBODY; COMPLEMENT ACTIVATION; BLISTER FORMATION; FOLIACEUS PF; B-CELLS; VULGARIS; AUTOANTIBODIES; AUTOIMMUNE;

D O I：

10.1016/j.jid.2021.04.040

中图分类号：

R75 [皮肤病学与性病学];

学科分类号：

100206 ;

摘要：

Pemphigus and pemphigoid are paradigms for understanding the mechanisms of antibody-mediated autoimmune disease in humans. In pemphigus, IgG4-predominant autoantibodies cause intraepidermal blistering by direct interference with desmoglein interactions and subsequent disruption of desmosomes and signaling pathways. In pemphigoid, IgG1, IgG4, and IgE autoantibodies against basement membrane zone antigens directly interfere with hemidesmosomal adhesion, activating complement and Fc receptor-mediated effector pathways. Unraveling disease mechanisms in pemphigus and pemphigoid has identified numerous opportunities for clinical trials, which hold promise to identify safer and more effective therapies for these potentially life-threatening diseases.

引用

页码：907 / 914

页数：8

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