title: Progressive respiratory failure in paraneoplastic pemphigus associated with chronic lymphocytic leukemia.
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.
authors.affiliation: Department of Dermatology, University Hospital, Bern, Switzerland. marcel.broennimann@insel.ch
authors.forename: M
authors.initials: M
authors.lastname: Brönnimann
authors.forename: V
authors.initials: V
authors.lastname: von Felbert
authors.forename: M
authors.initials: M
authors.lastname: Streit
authors.forename: T
authors.initials: T
authors.lastname: Hunziker
authors.forename: L R
authors.initials: LR
authors.lastname: Braathen
mesh_terms: D000368:Aged; D017809:Fatal Outcome; D006801:Humans; D015451:Leukemia, Lymphocytic, Chronic, B-Cell / Q000150:complications*; D008297:Male; D010257:Paraneoplastic Syndromes / Q000150:complications*; D010392:Pemphigus / Q000150:complications*; D012131:Respiratory Insufficiency / Q000209:etiology*