title: Five Japanese cases of antidesmoglein 1 antibody-positive and antidesmoglein 3 antibody-negative pemphigus with oral lesions.
abstract: BACKGROUND
  Oral mucosal lesions develop in pemphigus vulgaris, but not in pemphigus foliaceus. This clinical phenomenon is explained by the 'desmoglein (Dsg) compensation theory'. Dsg3 and Dsg1 are major autoantigens for pemphigus vulgaris and pemphigus foliaceus, respectively. Dsg3 is overexpressed and Dsg1 is weakly expressed on the oral mucosa. Thus, on the oral mucosa, suppression of Dsg3 function by anti-Dsg3 autoantibodies is not compensated by weakly expressed Dsg1 in pemphigus vulgaris, while suppression of Dsg1 function by anti-Dsg1 autoantibodies is perfectly compensated by richly expressed Dsg3 in pemphigus foliaceus.
  
  
  OBJECTIVE
  We present five Japanese patients with pemphigus who deviate from this theory, i.e. all patients showed oral lesions (three also had cutaneous lesions) and reacted only with Dsg1, but not with Dsg3, by enzyme-linked immunosorbent assay.
  
  
  METHODS
  To confirm whether the unique clinical phenotypes in our patients were due to a different immunological profile from that in classical pemphigus, we examined the reactivity of the patient sera by immunoprecipitation-immunoblotting analysis using five Dsg1/Dsg2 domain-swapped molecules.
  
  
  RESULTS
  The sera of two patients who had only oral lesions tended to react with the extracellular (EC) 5 domain of Dsg1, the domain that is considered nonpathogenic in classical pemphigus foliaceus. Sera of three patients with mucocutaneous lesions reacted with EC1 domain or with both EC1 and EC2 domains of Dsg1, like classical pemphigus foliaceus.
  
  
  CONCLUSIONS
  These results indicate that antigenic diversity of anti-Dsg1 antibodies in these patients may cause the unique oral mucosal and cutaneous lesions, although further studies are required to elucidate the pathomechanisms.
authors.affiliation: Department of Dermatology, Kurume University School of Medicine, Fukuoka, Japan.
authors.forename: H
authors.initials: H
authors.lastname: Koga
authors.forename: B
authors.initials: B
authors.lastname: Ohyama
authors.forename: D
authors.initials: D
authors.lastname: Tsuruta
authors.forename: N
authors.initials: N
authors.lastname: Ishii
authors.forename: T
authors.initials: T
authors.lastname: Hamada
authors.forename: T
authors.initials: T
authors.lastname: Dainichi
authors.forename: Y
authors.initials: Y
authors.lastname: Natsuaki
authors.forename: R
authors.initials: R
authors.lastname: Sogame
authors.forename: S
authors.initials: S
authors.lastname: Fukuda
authors.forename: T
authors.initials: T
authors.lastname: Karashima
authors.forename: J
authors.initials: J
authors.lastname: Tada
authors.forename: M
authors.initials: M
authors.lastname: Yamashiro
authors.forename: H
authors.initials: H
authors.lastname: Uezato
authors.forename: P T
authors.initials: PT
authors.lastname: Chan
authors.forename: T
authors.initials: T
authors.lastname: Hashimoto
mesh_terms: D000368:Aged; D001323:Autoantibodies / Q000378:metabolism*; D018076:DNA, Complementary; D051183:Desmoglein 1 / Q000276:immunology*; D051184:Desmoglein 3 / Q000276:immunology*; D005260:Female; D006801:Humans; D007070:Immunoglobulin A / Q000378:metabolism; D007074:Immunoglobulin G / Q000378:metabolism; D008297:Male; D008875:Middle Aged; D009059:Mouth Diseases / Q000276:immunology*; D009061:Mouth Mucosa; D010392:Pemphigus / Q000097:blood / Q000276:immunology*; D014162:Transfection / Q000379:methods
chemical_list: D001323:Autoantibodies; D018076:DNA, Complementary; D051183:Desmoglein 1; D051184:Desmoglein 3; D007070:Immunoglobulin A; D007074:Immunoglobulin G