title: Paraneoplastic pemphigus is associated with the DRB1*03 allele.
abstract: Pemphigus is a group of autoimmune blistering diseases caused by autoantibodies directed against keratinocyte adhesion molecules. Pemphigus vulgaris (PV) and pemphigus foliaceus (PF), in which autoantibodies bind, respectively, to desmoglein 3 and desmoglein 1, are strongly associated with HLA-class II DR4 and DR14 alleles. In paraneoplastic pemphigus (PNP), a rare variant associated with neoplasia, autoantibodies target proteins of the plakin family in addition to desmogleins 1 and 3. The presence of anti-desmoglein antibodies in all types of pemphigus raises the question of common molecular mechanisms of susceptibility, particularly similar MHC-class II allele associations, in the different forms of the disease. HLA-DRB1 typing was performed in 13 PNP patients and results were compared to those obtained from 84 healthy controls, 37 PV and 31 PF patients. Our data demonstrate a significant association of PNP with HLA-DRB1*03 allele which was found in 61.5% of the patients, whereas DRB1*04 and DRB1*14 appear not to be involved in PNP susceptibility. Therefore, the HLA-genetic background of PNP differs from that of other types of pemphigus, which suggests that distinct mechanism(s) initiate(s) the immunological response in this form of pemphigus.
authors.affiliation: INSERM U519 IFR 23, Rouen, France.
authors.forename: Philippe
authors.initials: P
authors.lastname: Martel
authors.forename: Pascale
authors.initials: P
authors.lastname: Loiseau
authors.forename: Pascal
authors.initials: P
authors.lastname: Joly
authors.forename: Marc
authors.initials: M
authors.lastname: Busson
authors.forename: Virginia
authors.initials: V
authors.lastname: Lepage
authors.forename: Hugo
authors.initials: H
authors.lastname: Mouquet
authors.forename: Philippe
authors.initials: P
authors.lastname: Courville
authors.forename: Béatrice
authors.initials: B
authors.lastname: Flageul
authors.forename: Dominique
authors.initials: D
authors.lastname: Charron
authors.forename: Philippe
authors.initials: P
authors.lastname: Musette
authors.forename: Danièle
authors.initials: D
authors.lastname: Gilbert
authors.forename: François
authors.initials: F
authors.lastname: Tron
mesh_terms: D006684:HLA-DR Antigens / Q000235:genetics*; D059811:HLA-DRB1 Chains; D006239:Haplotypes; D006801:Humans; D010257:Paraneoplastic Syndromes / Q000235:genetics / Q000276:immunology*; D010392:Pemphigus / Q000235:genetics / Q000276:immunology*
chemical_list: D006684:HLA-DR Antigens; D059811:HLA-DRB1 Chains