[Chronic lymphocytic leukemia revealed by atypic pemphigus].
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.
Voisard
J
J
Service de Dermatologie, Hôpital Haut-Lévêque, CHU de Bordeaux, avenue de Magellan, 33604 Pessac.
Beylot-Barry
M
M
Doutre
M
M
Vergier
B
B
Marrot
F
F
Joly
P
P
Beylot
C
C
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
D001324:Autoantigens; D015820:Cadherins; D051183:Desmoglein 1; D007074:Immunoglobulin G
Pemphigus atypique révélateur d'une leucémie lymphoïde chronique.