Immune response in pemphigus and beyond: progresses and emerging concepts

Di Zenzo, Giovanni; Amber, Kyle T; Sayar, Beyza; Müller, Eliane Jasmine; Borradori, Luca (2016). Immune response in pemphigus and beyond: progresses and emerging concepts. Seminars in immunopathology, 38(1), pp. 57-74. Springer 10.1007/s00281-015-0541-1

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Pemphigus vulgaris (PV) and pemphigus foliaceus (PF) are two severe autoimmune bullous diseases of the mucosae and/or skin associated with autoantibodies directed against desmoglein (Dsg) 3 and/or Dsg1. These two desmosomal cadherins, typifying stratified epithelia, are components of cell adhesion complexes called desmosomes and represent extra-desmosomal adhesion receptors. We herein review the advances in our understanding of the immune response underlying pemphigus, including human leucocyte antigen (HLA) class II-associated genetic susceptibility, characteristics of pathogenic anti-Dsg antibodies, antigenic mapping studies as well as findings about Dsg-specific B and T cells. The pathogenicity of anti-Dsg autoantibodies has been convincingly demonstrated. Disease activity and clinical phenotype correlate with anti-Dsg antibody titers and profile while passive transfer of anti-Dsg IgG from pemphigus patients' results in pemphigus-like lesions in neonatal and adult mice. Finally, adoptive transfer of splenocytes from Dsg3-knockout mice immunized with murine Dsg3 into immunodeficient mice phenotypically recapitulates PV. Although the exact pathogenic mechanisms leading to blister formation have not been fully elucidated, intracellular signaling following antibody binding has been found to be necessary for inducing cell-cell dissociation, at least for PV. These new insights not only highlight the key role of Dsgs in maintenance of tissue homeostasis but are expected to progressively change pemphigus management, paving the way for novel targeted immunologic and pharmacologic therapies.

Item Type:

Journal Article (Review Article)


05 Veterinary Medicine > Department of Infectious Diseases and Pathobiology (DIP) > Institute of Animal Pathology
04 Faculty of Medicine > Department of Dermatology, Urology, Rheumatology, Nephrology, Osteoporosis (DURN) > Clinic of Dermatology
04 Faculty of Medicine > Pre-clinic Human Medicine > BioMedical Research (DBMR) > Forschungsbereich Pathologie > Forschungsgruppe Dermatologie
05 Veterinary Medicine > Research Foci > DermFocus
05 Veterinary Medicine > Department of Infectious Diseases and Pathobiology (DIP)

UniBE Contributor:

Sayar, Beyza, Müller, Eliane Jasmine, Borradori, Luca


600 Technology > 610 Medicine & health
600 Technology > 630 Agriculture
500 Science > 570 Life sciences; biology








Barbara Gautschi-Steffen

Date Deposited:

15 Feb 2016 11:32

Last Modified:

05 Dec 2022 14:51

Publisher DOI:


PubMed ID:


Uncontrolled Keywords:

Acantholysis; Autoantibody; Cadherins; Cell-cell adhesion; Desmoglein; Desmosome; Fogo selvagem; Mature desmoglein; Monoclonal antibody; Pathogenesis; Pemphigus foliaceus; Pemphigus vulgaris; Precursor Dsg; Signaling pathways




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