Clinical and immunopathological spectrum of paraneoplastic pemphigus

Abstract

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.