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A case of pemphigus vulgaris showing reactivity with pemphigus antigens (Dsg1 and Dsg3) and desmocollins.

Abstract
Both pemphigus vulgaris antigen (PVA; Dsg3) and pemphigus foliaceus antigen (PFA; Dsg1) are members of the desmoglein subfamily of the cadherin supergene family. Another desmosomal cadherin, desmocollin, is occasionally recognized by certain pemphigus sera. We present a 38-year-old Japanese male who showed clinically and histopathologically typical features of pemphigus vulgaris, whose sera reacted with all PVA, PFA, and desmocollins using immunoblotting of both human epidermis and bovine snout epidermis. Studies using domain-specific fusion proteins of PFA and PVA suggested that this patient's serum reacted with the intracellular domain of PFA and the extracellular domain of PVA, the latter of which seems to be responsible for initiating the skin lesion. The patient's serum showed reactivity with human desmocollin and was shown to react with bovine Dsc2 fusion protein, further suggesting the significance of anti-desmocollin autoantibodies in pemphigus. These results indicate that certain pemphigus cases may produce antibodies against multiple antigen molecules, although the complex mechanism of the production of autoantibodies remains to be elucidated.
AuthorsT Hashimoto, M Amagai, K Watanabe, M Dmochowski, M A Chidgey, K K Yue, D R Garrod, T Nishikawa
JournalThe Journal of investigative dermatology (J Invest Dermatol) Vol. 104 Issue 4 Pg. 541-4 (Apr 1995) ISSN: 0022-202X [Print] United States
PMID7706774 (Publication Type: Case Reports, Journal Article, Research Support, Non-U.S. Gov't)
Chemical References
  • Autoantibodies
  • Autoantigens
  • Cadherins
  • Cytoskeletal Proteins
  • DSG1 protein, human
  • DSG3 protein, human
  • Desmocollins
  • Desmoglein 1
  • Desmoglein 3
  • Desmogleins
  • Desmoplakins
  • Dsg1a protein, mouse
  • Dsg3 protein, mouse
Topics
  • Adult
  • Animals
  • Autoantibodies (biosynthesis)
  • Autoantigens (immunology)
  • Cadherins (immunology)
  • Cattle
  • Cytoskeletal Proteins (immunology)
  • Desmocollins
  • Desmoglein 1
  • Desmoglein 3
  • Desmogleins
  • Desmoplakins
  • Desmosomes (immunology)
  • Humans
  • Immunoblotting
  • Male
  • Mice
  • Pemphigus (immunology)

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