S2k guidelines on the management of paraneoplastic pemphigus/paraneoplastic autoimmune multiorgan syndrome initiated by the European Academy of Dermatology and Venereology (EADV)

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 GrovesClaudia 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*

*Corresponding author for this work
8 Citations (Scopus)

Abstract

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. They have been initiated by the Task Force Autoimmune Blistering Diseases of the European Academy of Dermatology and Venereology with the contribution of physicians from all relevant disciplines. The degree of consent among all task force members was included. Results: Chronic severe mucositis and polymorphic skin lesions are clue clinical characteristics of PNP/PAMS. A complete assessment of the patient with suspected PNP/PAMS, requiring histopathological study and immunopathological investigations, including direct and indirect immunofluorescence, ELISA and, where available, immunoblotting/immunoprecipitation, is recommended to achieve a diagnosis of PNP/PAMS. Detection of anti-envoplakin antibodies and/or circulating antibodies binding to the rat bladder epithelium at indirect immunofluorescence is the most specific tool for the diagnosis of PNP/PAMS in a patient with compatible clinical and anamnestic features. Treatment of PNP/PAMS is highly challenging. Systemic steroids up to 1.5 mg/kg/day are recommended as first-line option. Rituximab is also recommended in patients with PNP/PAMS secondary to lymphoproliferative conditions but might also be considered in cases of PNP/PAMS associated with solid tumours. A multidisciplinary approach involving pneumologists, ophthalmologists and onco-haematologists is recommended for optimal management of the patients. Conclusions: These are the first European guidelines for the diagnosis and management of PNP/PAMS. Diagnostic criteria and therapeutic recommendations will require further validation by prospective studies.

Original languageEnglish
JournalJournal of the European Academy of Dermatology and Venereology
Volume37
Issue number6
Pages (from-to)1118-1134
Number of pages17
ISSN0926-9959
DOIs
Publication statusPublished - 06.2023

Research Areas and Centers

  • Academic Focus: Center for Infection and Inflammation Research (ZIEL)
  • Centers: Center for Research on Inflammation of the Skin (CRIS)

DFG Research Classification Scheme

  • 204-05 Immunology
  • 205-19 Dermatology

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