SearchDictionaryMobileLogin

A subset of pemphigus foliaceus patients exhibits pathogenic autoantibodies against both desmoglein-1 and desmoglein-3.

AbstractIn pemphigus vulgaris the major pathogenic antibody binds desmoglein-3, and mediates mucosal disease. Development of cutaneous disease is associated with acquisition of antibodies to desmoglein-1. In pemphigus foliaceus, and its endemic form, fogo selvagem by contrast, the major pathogenic antibody recognizes desmoglein-1 and mediates cutaneous disease only. In this study, we sought to determine the prevalence of antibodies to desmoglein-3 in patients with pemphigus foliaceus and fogo selvagem. We produced recombinant desmoglein-1 and desmoglein-3, and used them in highly sensitive and specific enzyme-linked immunosorbent assays, as well as immunoprecipitation assays. We detected antibodies to desmoglein-3 in 19 of 276 patients with pemphigus foliaceus and fogo selvagem, who had cutaneous disease only. We showed that these antibodies to desmoglein-3 could be absorbed in a concentration-dependent manner by desmoglein-3 but not by desmoglein-1. Also antibodies to desmoglein-1 could be absorbed in a concentration-dependent manner by desmoglein-1 but not desmoglein-3. This suggests that two separate species of antibody are present rather than one antibody capable of cross-reacting with both desmoglein-1 and desmoglein-3. Finally, it was shown that affinity-purified antibodies to desmoglein-3 from patients with pemphigus foliaceus and fogo selvagem induced a pemphigus vulgaris-like skin disease in mice by passive transfer. These results suggest that a subset of patients with pemphigus foliaceus and fogo selvagem have antibodies to desmoglein-3 that may be involved in the pathogenesis of their cutaneous disease.
AuthorsLuis A Arteaga, Philip S Prisayanh, Simon J P Warren, Zhi Liu, Luis A Diaz, Mong-Shang Lin, Cooperative Group on Fogo Selvagem Research (Affiliation: Department of Dermatology, University of North Carolina at Chapel Hill, 27599, USA.)
JournalThe Journal of investigative dermatology (J Invest Dermatol) Vol. 118 Issue 5 Pg. 806-11 (May 2002) ISSN: 0022-202X United States
PMID11982757 (Publication Type: Journal Article, Research Support, Non-U.S. Gov't, Research Support, U.S. Gov't, P.H.S.)
Chemical References
  • Autoantibodies
  • Cadherins
  • DSG3 protein, human
  • Desmoglein 1
  • Desmoglein 3
Topics
  • Animals
  • Animals, Newborn
  • Autoantibodies (blood, pharmacology)
  • Cadherins (immunology)
  • Desmoglein 1
  • Desmoglein 3
  • Enzyme-Linked Immunosorbent Assay
  • Humans
  • Mice
  • Mice, Inbred BALB C
  • Pemphigus (immunology)
  • Sensitivity and Specificity
  • Skin (immunology)