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Fatal paraneoplastic pemphigus associated with a mediastinal tumor
Author(s) -
Pfistershammer Katharina,
Minimair Gerald,
Födinger Dagmar,
Czerny Martin,
Zauner Christian,
Pieczkowski Friederike,
Stingl Georg,
Karlhofer Franz M.
Publication year - 2009
Publication title -
jddg: journal der deutschen dermatologischen gesellschaft
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.463
H-Index - 60
eISSN - 1610-0387
pISSN - 1610-0379
DOI - 10.1111/j.1610-0387.2008.06939.x
Subject(s) - paraneoplastic pemphigus , medicine , histopathology , dermatology , rash , pathology , pemphigus , sarcoma , immunology , autoantibody , antibody
Summary 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.