<p>Pemphigus is a group of rare autoimmune blistering disorders affecting the skin and mucosal surfaces, caused by pathogenic immunoglobulin G (IgG) autoantibodies targeting desmosomal cadherins, specifically desmoglein-1 and desmoglein-3, which are key components of desmosomes. There are two main forms of pemphigus: pemphigus vulgaris (PV) and pemphigus foliaceus (PF), with PV being the most common. Pemphigus can be life-threatening owing to the progressive loss of the epidermal and epithelial barrier function. However, the introduction of rituximab, an anti-CD20 monoclonal antibody, has significantly improved treatment outcomes in pemphigus. This review provides a comprehensive overview of the current treatment landscape for pemphigus, highlighting both established and emerging therapeutic approaches.</p>

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Emerging Treatments in Pemphigus: Is Healing an Achievable Goal?

  • Barbara Horváth,
  • Marjolein A. J. Hiel,
  • Anne-Lise Strandmoe,
  • Joost M. Meijer

摘要

Pemphigus is a group of rare autoimmune blistering disorders affecting the skin and mucosal surfaces, caused by pathogenic immunoglobulin G (IgG) autoantibodies targeting desmosomal cadherins, specifically desmoglein-1 and desmoglein-3, which are key components of desmosomes. There are two main forms of pemphigus: pemphigus vulgaris (PV) and pemphigus foliaceus (PF), with PV being the most common. Pemphigus can be life-threatening owing to the progressive loss of the epidermal and epithelial barrier function. However, the introduction of rituximab, an anti-CD20 monoclonal antibody, has significantly improved treatment outcomes in pemphigus. This review provides a comprehensive overview of the current treatment landscape for pemphigus, highlighting both established and emerging therapeutic approaches.