Clinical and immunopathological spectrum of paraneoplastic pemphigus

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.

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.

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.

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.