Pemphigus

Michael Kasperkiewicz, Christoph T. Ellebrecht, Hayato Takahashi, Jun Yamagami, Detlef Zillikens, Aimee S. Payne, Masayuki Amagai

Research output: Contribution to journalReview article

92 Citations (Scopus)

Abstract

Pemphigus is a group of IgG-mediated autoimmune diseases of stratified squamous epithelia, such as the skin and oral mucosa, in which acantholysis (the loss of cell adhesion) causes blisters and erosions. Pemphigus has three major subtypes: Pemphigus vulgaris, pemphigus foliaceus and paraneoplastic pemphigus. IgG autoantibodies are characteristically raised against desmoglein 1 and desmoglein 3, which are cell-cell adhesion molecules found in desmosomes. The sites of blister formation can be physiologically explained by the anti-desmoglein autoantibody profile and tissue-specific expression pattern of desmoglein isoforms. The pathophysiological roles of T cells and B cells have been characterized in mouse models of pemphigus and patients, revealing insights into the mechanisms of autoimmunity. Diagnosis is based on clinical manifestations and confirmed with histological and immunochemical testing. The current first-line treatment is systemic corticosteroids and adjuvant therapies, including immunosuppressive agents, intravenous immunoglobulin and plasmapheresis. Rituximab, a monoclonal antibody against CD20+ B cells, is a promising therapeutic option that may soon become first-line therapy. Pemphigus is one of the best-characterized human autoimmune diseases and provides an ideal paradigm for both basic and clinical research, especially towards the development of antigen-specific immune suppression treatments for autoimmune diseases.

Original languageEnglish
Article number17026
JournalNature Reviews Disease Primers
Volume3
DOIs
Publication statusPublished - 2017 May 11

Fingerprint

Pemphigus
Desmogleins
Autoimmune Diseases
Blister
Autoantibodies
B-Lymphocytes
Desmoglein 3
Immunoglobulin G
Desmoglein 1
Acantholysis
Therapeutics
Desmosomes
Plasmapheresis
Intravenous Immunoglobulins
Mouth Mucosa
Cell Adhesion Molecules
Immunosuppressive Agents
Autoimmunity
Cell Adhesion
Adrenal Cortex Hormones

ASJC Scopus subject areas

  • Medicine(all)

Cite this

Kasperkiewicz, M., Ellebrecht, C. T., Takahashi, H., Yamagami, J., Zillikens, D., Payne, A. S., & Amagai, M. (2017). Pemphigus. Nature Reviews Disease Primers, 3, [17026]. https://doi.org/10.1038/nrdp.2017.26

Pemphigus. / Kasperkiewicz, Michael; Ellebrecht, Christoph T.; Takahashi, Hayato; Yamagami, Jun; Zillikens, Detlef; Payne, Aimee S.; Amagai, Masayuki.

In: Nature Reviews Disease Primers, Vol. 3, 17026, 11.05.2017.

Research output: Contribution to journalReview article

Kasperkiewicz, M, Ellebrecht, CT, Takahashi, H, Yamagami, J, Zillikens, D, Payne, AS & Amagai, M 2017, 'Pemphigus', Nature Reviews Disease Primers, vol. 3, 17026. https://doi.org/10.1038/nrdp.2017.26
Kasperkiewicz M, Ellebrecht CT, Takahashi H, Yamagami J, Zillikens D, Payne AS et al. Pemphigus. Nature Reviews Disease Primers. 2017 May 11;3. 17026. https://doi.org/10.1038/nrdp.2017.26
Kasperkiewicz, Michael ; Ellebrecht, Christoph T. ; Takahashi, Hayato ; Yamagami, Jun ; Zillikens, Detlef ; Payne, Aimee S. ; Amagai, Masayuki. / Pemphigus. In: Nature Reviews Disease Primers. 2017 ; Vol. 3.
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