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Progressive respiratory failure in paraneoplastic pemphigus associated with chronic lymphocytic leukemia
M Brönnimann1, V von Felbert, M Streit
1Department of Dermatology, University Hospital, Bern, Switzerland. marcel.broennimann@insel.ch
Abstract:
A 70-year-old Caucasian man with chronic lymphocytic leukemia suffered from widespread, histologically proven cutaneous lichen planus responding to topical corticosteroids. 2 years later, he presented with painful erosive stomatitis and increasing dyspnea. Histology, direct and indirect immunofluorescence were diagnostic for paraneoplastic pemphigus. A full diagnostic workup could not disclose the cause of the progressive respiratory insufficiency. Despite aggressive treatment of the lymphocytic leukemia and the paraneoplastic pemphigus, the patient died 3 months after diagnosis. Paraneoplastic pemphigus may lead to pulmonary failure which is refractory to treatment and has a fatal outcome.
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