PMID- 29313220
OWN - NLM
STAT- MEDLINE
DCOM- 20180816
LR  - 20210331
IS  - 1559-0267 (Electronic)
IS  - 1080-0549 (Linking)
VI  - 54
IP  - 1
DP  - 2018 Feb
TI  - Pemphigus: a Comprehensive Review on Pathogenesis, Clinical Presentation and 
      Novel Therapeutic Approaches.
PG  - 1-25
LID - 10.1007/s12016-017-8662-z [doi]
AB  - Pemphigus is a group of rare, potentially devastating autoimmune diseases of the 
      skin and mucous membranes with high morbidity and potentially lethal outcome. The 
      major clinical variant, pemphigus vulgaris (PV) is caused by a loss of 
      intercellular adhesion of epidermal keratinocytes which is induced by IgG 
      autoantibodies against components of desmosomes. Specifically, IgG against the 
      desmosomal adhesion proteins, desmoglein 3 (Dsg3) and desmoglein 1 (Dsg1), 
      preferentially target their ectodomains which are presumably critical for the 
      transinteraction and signalling function of these adhesion molecules. There is a 
      close immunogenetic association of PV with the human leukocyte antigen (HLA) 
      class II alleles, HLA-DRB1*04:02 and HLA-DQB1*05:03. These have been shown to be 
      critical for the presentation of immunodominant peptides to autoreactive CD4+ T 
      helper cells. The importance of autoaggressive T-B cell interaction in the 
      induction of pathogenic IgG autoantibodies which directly cause epidermal loss of 
      adhesion has been demonstrated both clinically (by the use of the anti-CD20 
      monoclonal antibody rituximab) and experimentally (in PV mouse models). The 
      strong association of clinically active pemphigus with autoantibodies of the 
      IgG(4) and IgE subclasses strongly suggests that T helper 2 cells are critical 
      regulators of the immune pathogenesis of pemphigus. Novel therapeutic approaches 
      target autoreactive T and B cells to specifically interfere with the T 
      cell-dependent activation of B cells leading to the generation of 
      autoantibody-producing plasma cells. Our improved understanding of the 
      autoantibody-driven effector phase of pemphigus has led to the introduction of 
      novel therapies that target pathogenic autoantibodies such as immunoadsorption 
      and drugs that block pathogenic autoantibody-induced cell signalling events.
FAU - Pollmann, Robert
AU  - Pollmann R
AD  - Department of Dermatology and Allergology, Philipps-University, Baldingerstrasse, 
      D-35043, Marburg, Germany.
FAU - Schmidt, Thomas
AU  - Schmidt T
AD  - Department of Dermatology and Allergology, Philipps-University, Baldingerstrasse, 
      D-35043, Marburg, Germany.
FAU - Eming, Rudiger
AU  - Eming R
AD  - Department of Dermatology and Allergology, Philipps-University, Baldingerstrasse, 
      D-35043, Marburg, Germany.
FAU - Hertl, Michael
AU  - Hertl M
AD  - Department of Dermatology and Allergology, Philipps-University, Baldingerstrasse, 
      D-35043, Marburg, Germany. hertl@med.uni-marburg.de.
LA  - eng
GR  - FOR 2497/Deutsche Forschungsgemeinschaft/
GR  - FOR 2497/Deutsche Forschungsgemeinschaft/
PT  - Journal Article
PT  - Review
PL  - United States
TA  - Clin Rev Allergy Immunol
JT  - Clinical reviews in allergy & immunology
JID - 9504368
RN  - 0 (Autoantibodies)
RN  - 0 (Autoantigens)
RN  - 0 (Desmoglein 1)
RN  - 0 (Desmoglein 3)
RN  - 0 (Immunoglobulin G)
RN  - 37341-29-0 (Immunoglobulin E)
SB  - IM
MH  - Animals
MH  - Autoantibodies/metabolism
MH  - Autoantigens/*immunology
MH  - Autoimmune Diseases/*immunology
MH  - Desmoglein 1/*immunology
MH  - Desmoglein 3/*immunology
MH  - Desmosomes/metabolism
MH  - Epidermis/*pathology
MH  - Humans
MH  - Immunoglobulin E/metabolism
MH  - Immunoglobulin G/metabolism
MH  - Keratinocytes/*physiology
MH  - Pemphigus/*immunology
MH  - Signal Transduction
OTO - NOTNLM
OT  - Autoantibodies
OT  - Diagnostics
OT  - Mouse models
OT  - Pathogenesis
OT  - Pemphigus
OT  - Pemphigus foliaceus
OT  - Pemphigus vulgaris
OT  - Treatment
EDAT- 2018/01/10 06:00
MHDA- 2018/08/17 06:00
CRDT- 2018/01/10 06:00
PHST- 2018/01/10 06:00 [pubmed]
PHST- 2018/08/17 06:00 [medline]
PHST- 2018/01/10 06:00 [entrez]
AID - 10.1007/s12016-017-8662-z [pii]
AID - 10.1007/s12016-017-8662-z [doi]
PST - ppublish
SO  - Clin Rev Allergy Immunol. 2018 Feb;54(1):1-25. doi: 10.1007/s12016-017-8662-z.