Emiliano Antiga, Rikke Bech, Roberto Maglie, Giovanni Genovese, Luca Borradori, Barbara Bockle, Marzia Caproni, Frédéric Caux, Nisha Suyien Chandran, Alberto Corrà, Francesco D'Amore, Maryam Daneshpazhooh, Dipankar De, Dario Didona, Marian Dmochowski, Kossara Drenovska, Jan Ehrchen, Claudio Feliciani, Matthias Goebeler, Richard Groves, Claudia Günther, Sanjeev Handa, Silke C Hofmann, Barbara Horvath, Dimitrios Ioannidis, Hana Jedlickova, Cezary Kowalewski, Khalaf Kridin, Pascal Joly, Yen Loo Lim, Branka Marinovic, Emanual Maverakis, Joost Meijer, Aikaterini Patsatsi, Carlo Pincelli, Catherine Prost, Jane Setterfield, Eli Sprecher, Dusan Skiljevic, Kaisa Tasanen, Soner Uzun, Nina Van Beek, Snejina Vassileva, Artem Vorobyev, Igor Vujic, Gang Wang, Mingyue Wang, Katarzyna Wozniak, Savas Yayli, Giovanna Zambruno, Takashi Hashimoto, Enno Schmidt, José Manuel Mascarò, Angelo Valerio Marzano
BACKGROUND: Paraneoplastic pemphigus (PNP), also called paraneoplastic autoimmune multiorgan syndrome (PAMS), is a rare autoimmune disease with mucocutaneous and multi-organ involvement. PNP/PAMS is typically associated with lymphoproliferative or haematological malignancies, and less frequently with solid malignancies. The mortality rate of PNP/PAMS is elevated owing to the increased risk of severe infections and disease-associated complications, such as bronchiolitis obliterans. OBJECTIVES: These guidelines summarize evidence-based and expert-based recommendations (S2k level) for the clinical characterization, diagnosis and management of PNP/PAMS...
March 25, 2023: Journal of the European Academy of Dermatology and Venereology: JEADV