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Paraneoplastic pemphigus with eosinophilic spongiosis and autoantibodies against desmocollins 2 and 3.

Abstract
Paraneoplastic pemphigus (PNP) is an autoimmune blistering disease associated with neoplasms, typically lymphoproliferative disorders. PNP is characterized clinically by painful erosive stomatitis and polymorphous skin lesions. Histopathological findings are also very varied, and include lichen planus-like and pemphigus-like changes. These polymorphic clinicopathological findings are probably due to the complex pathogenic mechanism, in which both cellular and humoral immunity are implicated. Eosinophilic spongiosis, although infrequent, can be found with pemphigus herpetiformis and bullous pemphigoid, although this association has not been established in PNP. The presence of autoantibodies against envoplakin and periplakin in PNP has been reported, but autoantibodies against desmocollins (Dscs) have been found in only a very few cases of PNP, probably due to the lack of studies on such associations. We report the first case, to our knowledge, of PNP with eosinophilic spongiosis as the initial histopathological finding, and presence of autoantibodies to Dsc2 and Dsc3.
AuthorsE Gallo, P García-Martín, J Fraga, K Teye, H Koga, T Hashimoto, A García-Diez
JournalClinical and experimental dermatology (Clin Exp Dermatol) Vol. 39 Issue 3 Pg. 323-6 (Apr 2014) ISSN: 1365-2230 [Electronic] England
PMID24635070 (Publication Type: Case Reports, Journal Article)
Copyright© 2014 British Association of Dermatologists.
Chemical References
  • Autoantibodies
  • DSC2 protein, human
  • DSC3 protein, human
  • Desmocollins
Topics
  • Aged, 80 and over
  • Autoantibodies (immunology)
  • Desmocollins (immunology)
  • Eosinophilia (pathology)
  • Female
  • Humans
  • Paraneoplastic Syndromes (immunology)
  • Pemphigoid, Bullous (immunology)

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