title: Are clinical phenotype and autoantibody profile always concordant in pemphigus? A study in a cohort of pemphigus patients.
abstract: BACKGROUND
  The clinical phenotype of different forms of pemphigus is reportedly defined by the anti-desmoglein (Dsg) autoantibody profile. In routine practice, however, this is not always the case.
  
  
  OBJECTIVE
  To verify the relationship between the anti-Dsg1 and -3 autoantibody profiles and titers on the one hand and the clinical phenotype and disease activity on the other.
  
  
  METHODS
  we followed-up clinically and serologically 20 pemphigus patients, including 3 mucosal pemphigus (mPV), 9 mucocutaneous pemphigus (mcPV), and 8 cutaneous pemphigus (PF).
  
  
  RESULTS
  We found that the cutaneous and/or mucosal involvement and the autoantibody profile were only concordant in mPV patients. On the contrary, in other clinical forms this correlation was often absent.
  
  
  CONCLUSIONS
  1) The discrepancy between autoantibody profile and the clinical phenotype, at least in PF patients, appears to be due to non-pathogenic anti-Dsg3 antibodies; 2) in a proportion of patients the relationship between the Dsg1 and Dsg3 ELISA titers and the disease severity was absent; 3) in some patients, the anti-Dsg1 and -3 autoantibodies were lacking at diagnosis, suggesting a role of other antigens in the pathogenesis of the disease and, lastly, 4) the pure cutaneous and mucosal forms tend to respond more efficiently to the therapy than the mucocutaneous forms and have a persistent response.
authors: DISSAL, Section of Dermatology, IRCCS, Azienda Ospedaliera San Martino-IST, Via Pastore 1, Genoa 16132, Italy. emanuele.cozzani@unige.it
authors: Emanuele
authors: E
authors: Cozzani
authors: Giovanni
authors: G
authors: Di Zenzo
authors: Silvia
authors: S
authors: Riva
authors: Valentina
authors: V
authors: Calabresi
authors: Francesco
authors: F
authors: Sera
authors: Massimo
authors: M
authors: Drosera
authors: Aurora
authors: A
authors: Parodi
mesh_terms: D000368:Aged; D000704:Analysis of Variance; D001323:Autoantibodies / Q000097:blood*; D051183:Desmoglein 1 / Q000276:immunology*; D051184:Desmoglein 3 / Q000276:immunology*; D005260:Female; D005500:Follow-Up Studies; D005938:Glucocorticoids / Q000627:therapeutic use; D006801:Humans; D008297:Male; D010392:Pemphigus / Q000145:classification / Q000188:drug therapy / Q000276:immunology*; D010641:Phenotype; D012189:Retrospective Studies; D012720:Severity of Illness Index; D016896:Treatment Outcome
chemical_list: D001323:Autoantibodies; D051183:Desmoglein 1; D051184:Desmoglein 3; D005938:Glucocorticoids
keywords: ELISA; autoantibody profile; clinical phenotype; desmoglein; diagnosis; pemphigus