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Paraneoplastic pemphigus with eosinophilic spongiosis and autoantibodies against desmocollins 2 and 3
Author(s) -
Gallo E.,
GarcíaMartín P.,
Fraga J.,
Teye K.,
Koga H.,
Hashimoto T.,
GarcíaDiez A.
Publication year - 2014
Publication title -
clinical and experimental dermatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.587
H-Index - 78
eISSN - 1365-2230
pISSN - 0307-6938
DOI - 10.1111/ced.12296
Subject(s) - spongiosis , paraneoplastic pemphigus , medicine , autoantibody , pemphigus , pemphigus foliaceus , immunology , eosinophilic , pathology , dermatology , antibody
Summary 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 ( D scs) 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 D sc2 and D sc3.