Paraneoplastic pemphigus. A detailed case series from the Netherlands revealing atypical cases

BACKGROUND: Paraneoplastic pemphigus (PNP) is an extremely rare life-threatening blistering autoimmune disease that is associated with an underlying neoplasm. There is a set diagnostic criterion for PNP, which is primarily based on a severe stomatitis and the detection of specific antibodies against envoplakin, periplakin and alpha-2-macroglobulin-like protein 1. However, it has becoming increasingly evident that there are patients with PNP that do not meet all the diagnostic criteria requirements. OBJECTIVES: The aim of this study was to analyse our cohort of Dutch patients and to define the... Mehr ...