title: [Chronic lymphocytic leukemia revealed by atypic pemphigus].
abstract: BACKGROUND
  Bullous diseases are occasionally reported during chronic lymphocytic leukemia. We report the case of a woman in which a bullous disease of difficult nosologic classification has revealed chronic lymphocytic leukemia.
  
  
  METHODS
  A 57-year-old woman, well-known for a rheumatoid arthritis, developed a bullous eruption. It was associated with voluminous lymphadenopathies, and laboratory investigations revealed a chronic lymphocytic leukemia. Clinicopathological aspect and immunofluorescence studies argued for a paraneoplastic pemphigus but immunoblot showed only antibodies against desmoglein 1.
  
  
  CONCLUSIONS
  Paraneoplastic pemphigus is an autoimmune bullous disease defined by the criteria of Anhalt, including autoantibodies mostly directed against cytoplasmic proteins of the plakin family. Our case is unusual for the absence of these antibodies and for the presence of antibodies directed against a cell surface target, desmoglein 1. Such a case confirms the overlapping auto-immune responses in pemphigus.
authors.affiliation: Service de Dermatologie, Hôpital Haut-Lévêque, CHU de Bordeaux, avenue de Magellan, 33604 Pessac.
authors.forename: J
authors.initials: J
authors.lastname: Voisard
authors.forename: M
authors.initials: M
authors.lastname: Beylot-Barry
authors.forename: M
authors.initials: M
authors.lastname: Doutre
authors.forename: B
authors.initials: B
authors.lastname: Vergier
authors.forename: F
authors.initials: F
authors.lastname: Marrot
authors.forename: P
authors.initials: P
authors.lastname: Joly
authors.forename: C
authors.initials: C
authors.lastname: Beylot
mesh_terms: D001172:Arthritis, Rheumatoid / Q000150:complications; D001324:Autoantigens / Q000032:analysis; D001327:Autoimmune Diseases / Q000276:immunology; D015820:Cadherins / Q000032:analysis; D051183:Desmoglein 1; D005260:Female; D019085:Fluorescent Antibody Technique, Direct; D006801:Humans; D015151:Immunoblotting; D007074:Immunoglobulin G / Q000032:analysis; D015451:Leukemia, Lymphocytic, Chronic, B-Cell / Q000150:complications*; D008875:Middle Aged; D010257:Paraneoplastic Syndromes / Q000209:etiology* / Q000276:immunology; D010392:Pemphigus / Q000209:etiology* / Q000276:immunology
chemical_list: D001324:Autoantigens; D015820:Cadherins; D051183:Desmoglein 1; D007074:Immunoglobulin G
vernacular_title: Pemphigus atypique révélateur d'une leucémie lymphoïde chronique.