title: Fatal paraneoplastic pemphigus associated with a mediastinal tumor.
abstract: Paraneoplastic pemphigus (PNP) is a rare life-threatening autoimmune bullous skin disease which is an obligate paraneoplasma. A 34-year-old woman presented with recalcitrant stomatitis and a generalized lichenoid rash. A diagnosis of PNP was established based on clinical findings, immunofluorescence, histopathology and biochemistry. A localized mediastinal mass was found with CT imaging and excised. The histologic diagnosis was dendritic cell sarcoma. Despite removal of tumor and immunosuppressive therapy, the PNP progressed rapidly and the patient died of septic multiorgan failure.
authors.affiliation: Department of Dermatology, Division of Immunodermatology and Infectious Skin Diseases, Medical University of Vienna, Austria.
authors.forename: Katharina
authors.initials: K
authors.lastname: Pfistershammer
authors.forename: Gerald
authors.initials: G
authors.lastname: Minimair
authors.forename: Dagmar
authors.initials: D
authors.lastname: Födinger
authors.forename: Martin
authors.initials: M
authors.lastname: Czerny
authors.forename: Christian
authors.initials: C
authors.lastname: Zauner
authors.forename: Friederike
authors.initials: F
authors.lastname: Pieczkowski
authors.forename: Georg
authors.initials: G
authors.lastname: Stingl
authors.forename: Franz M
authors.initials: FM
authors.lastname: Karlhofer
mesh_terms: D000328:Adult; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D008479:Mediastinal Neoplasms / Q000150:complications* / Q000175:diagnosis*; D010257:Paraneoplastic Syndromes / Q000150:complications* / Q000175:diagnosis*; D010392:Pemphigus / Q000150:complications* / Q000175:diagnosis*