Humoral epitope spreading in autoimmune bullous diseases

Dario Didona, Giovanni Di Zenzo

Research output: Contribution to journalReview articlepeer-review


Autoimmune blistering diseases are characterized by autoantibodies against structural adhesion proteins of the skin and mucous membranes. Extensive characterization of their autoantibody targets has improved understanding of pathogenesis and laid the basis for the study of antigens/epitopes diversification, a process termed epitope spreading (ES). In this review, we have reported and discussed ES phenomena in autoimmune bullous diseases and underlined their functional role in disease pathogenesis. A functional ES has been proposed: (1) in bullous pemphigoid patients and correlates with the initial phase of the disease, (2) in pemphigus vulgaris patients with mucosal involvement during the clinical transition to a mucocutaneous form, (3) in endemic pemphigus foliaceus, underlining its role in disease pathogenesis, and (4) in numerous cases of disease transition associated with an intermolecular diversification of immune response. All these findings could give useful information to better understand autoimmune disease pathogenesis and to design antigen/epitope specific therapeutic approaches.

Original languageEnglish
Article number779
JournalFrontiers in Immunology
Issue numberAPR
Publication statusPublished - Apr 17 2018


  • Antigen
  • Autoantibody
  • BP180
  • Desmoglein
  • Epitope
  • Epitope spreading
  • Pathogenesis
  • Specific therapy

ASJC Scopus subject areas

  • Immunology and Allergy
  • Immunology


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