4.8 Review

The relevance of complement in pemphigoid diseases: A critical appraisal

Journal

FRONTIERS IN IMMUNOLOGY
Volume 13, Issue -, Pages -

Publisher

FRONTIERS MEDIA SA
DOI: 10.3389/fimmu.2022.973702

Keywords

complement; relevance; pemphigoid; bullous pemphigoid; BP; EBA; MMP; pathophysiology

Categories

Funding

  1. DFG [454193335]
  2. CRU [303 P7]
  3. Research Training Group Autoimmune Pre-Disease [GRK 2633]
  4. Schleswig-Holstein Excellence-Chair Program from the State of Schleswig Holstein
  5. Excellence Cluster EXC 2167 Precision Medicine in Chronic Inflammation [TI-4]
  6. Sinergia Unravel principles of self-organization in injured tissue from the Swiss National Science Foundation [CRSII5_202301/1]
  7. Swiss National Science Foundation (SNF) [CRSII5_202301] Funding Source: Swiss National Science Foundation (SNF)

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Pemphigoid diseases are chronic inflammatory skin diseases characterized by blistering and autoantibodies. Complement plays an important role in these diseases, but recent studies have shown that inflammation can occur independently of complement.
Pemphigoid diseases are autoimmune chronic inflammatory skin diseases, which are characterized by blistering of the skin and/or mucous membranes, and circulating and tissue-bound autoantibodies. The well-established pathomechanisms comprise autoantibodies targeting various structural proteins located at the dermal-epidermal junction, leading to complement factor binding and activation. Several effector cells are thus attracted and activated, which in turn inflict characteristic tissue damage and subepidermal blistering. Moreover, the detection of linear complement deposits in the skin is a diagnostic hallmark of all pemphigoid diseases. However, recent studies showed that blistering might also occur independently of complement. This review reassesses the importance of complement in pemphigoid diseases based on current research by contrasting and contextualizing data from in vitro, murine and human studies.

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