Clinical and immunopathological spectrum of paraneoplastic pemphigus
Article first published online: 18 FEB 2010
© The Authors • Journal compilation © Blackwell Verlag GmbH, Berlin
JDDG: Journal der Deutschen Dermatologischen Gesellschaft
Volume 8, Issue 8, pages 598–605, August 2010
How to Cite
Zimmermann, J., Bahmer, F., Rose, C., Zillikens, D. and Schmidt, E. (2010), Clinical and immunopathological spectrum of paraneoplastic pemphigus. JDDG: Journal der Deutschen Dermatologischen Gesellschaft, 8: 598–605. doi: 10.1111/j.1610-0387.2010.07380.x
- Issue published online: 20 JUL 2010
- Article first published online: 18 FEB 2010
- Submitted: 3.11.2009 | Accepted: 23.12.2009
Background: Paraneoplastic pemphigus (PNP) is a rare life-threatening autoimmune bullous disorder. The clinical picture is often polymorphous and blisters may be absent. Autoantibodies are directed against several target antigens, including plakins and desmogleins. PNP has a high mortality rate that does not directly depend on the malignancy of the neoplasm. No generally accepted diagnostic criteria for PNP have been defined.
Patients and Methods: On the basis of four selected PNP patients and the relevant literature, the spectrum of clinical and immunopathological findings as well as pathogenesis and treatment options of PNP are presented.
Results: In addition to a neoplasm, severe stomatitis and the presence of autoantibodies against periplakin and envoplakin are characteristic for PNP. Based on the presented data, diagnostic criteria are proposed.
Conclusions: Knowledge of the polymorphous clinical picture and the complex autoantibody response is essential for an early diagnosis of PNP which has implications for both prognosis and rapid initiation of treatment.