Pfistershammer Katharina, Minimair Gerald, Födinger Dagmar, Czerny Martin, Zauner Christian, Pieczkowski Friederike, Stingl Georg, Karlhofer Franz M
Department of Dermatology, Division of Immunodermatology and Infectious Skin Diseases, Medical University of Vienna, Austria.
J Dtsch Dermatol Ges. 2009 Apr;7(4):356-9. doi: 10.1111/j.1610-0387.2008.06939.x. Epub 2008 Dec 12.
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.
副肿瘤性天疱疮（PNP）是一种罕见的、危及生命的自身免疫性大疱性皮肤病，是一种必然与肿瘤相关的疾病。一名34岁女性出现顽固性口腔炎和全身性苔藓样皮疹。根据临床表现、免疫荧光、组织病理学和生物化学检查确诊为PNP。CT成像发现一个局限性纵隔肿块并将其切除。组织学诊断为树突状细胞肉瘤。尽管切除了肿瘤并进行了免疫抑制治疗，但PNP仍迅速进展，患者死于感染性多器官功能衰竭。
