Chronic lymphocytic leukemia revealed by atypic pemphigus.

Background. Bullous diseases are occasionally reported during chronic lymph ocytic leukemia. We report the case of a woman in which a bullous disease o f difficult nosologic classification has revealed chronic lymphocytic leuke mia. Case report. A 57 year-old woman, well-known for a rheumatoid arthritis, de veloped a bullous eruption. It was associated with voluminous lymphadenopat hies, and laboratory investigations revealed a chronic lymphocytic leukemia . Clinicopathological aspect and immunofluorescence studies argued for a pa raneoplastic pemphigus but immunoblot showed only antibodies against desmog lein 1. Discussion. Paraneoplastic pemphigus is an autoimmune bullous disease defin ed by the criteria of Anhalt, including autoantibodies mostly directed agai nst cytoplasmic proteins of the plakin family. Our case is unusual for the absence of these antibodies and for the presence of antibodies directed aga inst a cell surface target, desmoglein 1. Such a case confirms the overlapp ing auto-immune responses in pemphigus.