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S2k guidelines on the management of paraneoplastic pemphigus/paraneoplastic autoimmune multiorgan syndrome initiated by the European Academy of Dermatology and Venereology (EADV)
Journal of the European Academy of Dermatology and Venereology, 37 (2023), 6; 1118-1134. https://doi.org/10.1111/jdv.18931
Antiga, Emiliano; Bech, Rikke; Maglie, Roberto; Genovese, Giovanni; Borradori, Luca; Bockle, Barbara; Caproni, Marzia; Caux, Frédéric; Chandran, Nisha Suyien; Corrà, Alberto; D'Amore, Francesco; Daneshpazhooh, Maryam; De, Dipankar; Didona, Dario; Dmochowski, Marian; Drenovska, Kossara; Ehrchen, Jan; Feliciani, Claudio; Goebeler, Matthias; Groves, Richard; Günther, Claudia; Handa, Sanjeev; Hofmann, Silke C.; Horvath, Barbara; Ioannidis, Dimitrios; Jedlickova, Hana; Kowalewski, Cezary; Kridin, Khalaf; Joly, Pascal; Lim, Yen Loo; Marinović, Branka; Maverakis, Emanual; Meijer, Joost; Patsatsi, Aikaterini; Pincelli, Carlo; Prost, Catherine; Setterfield, Jane; Sprecher, Eli; Skiljevic, Dusan; Tasanen, Kaisa; Uzun, Soner; Van Beek, Nina; Vassileva, Snejina; Vorobyev, Artem; Vujic, Igor; Wang, Gang; Wang, Mingyue; Wozniak, Katarzyna; Yayli, Savas; Zambruno, Giovanna; Hashimoto, Takashi; Schmidt, Enno; Mascarò, José Manuel; Marzano, Angelo Valerio More authors...

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Antiga, E., Bech, R., Maglie, R., Genovese, G., Borradori, L., Bockle, B. ... Marzano, A. V. (2023). S2k guidelines on the management of paraneoplastic pemphigus/paraneoplastic autoimmune multiorgan syndrome initiated by the European Academy of Dermatology and Venereology (EADV). Journal of the European Academy of Dermatology and Venereology, 37. (6), 1118-1134. doi: 10.1111/jdv.18931

Antiga, Emiliano, et al. "S2k guidelines on the management of paraneoplastic pemphigus/paraneoplastic autoimmune multiorgan syndrome initiated by the European Academy of Dermatology and Venereology (EADV)." Journal of the European Academy of Dermatology and Venereology, vol. 37, no. 6, 2023, pp. 1118-1134. https://doi.org/10.1111/jdv.18931

Antiga, Emiliano, Rikke Bech, Roberto Maglie, Giovanni Genovese, Luca Borradori, Barbara Bockle, Marzia Caproni, et al. "S2k guidelines on the management of paraneoplastic pemphigus/paraneoplastic autoimmune multiorgan syndrome initiated by the European Academy of Dermatology and Venereology (EADV)." Journal of the European Academy of Dermatology and Venereology 37, no. 6 (2023): 1118-1134. https://doi.org/10.1111/jdv.18931

Antiga, E., et al. (2023) 'S2k guidelines on the management of paraneoplastic pemphigus/paraneoplastic autoimmune multiorgan syndrome initiated by the European Academy of Dermatology and Venereology (EADV)', Journal of the European Academy of Dermatology and Venereology, 37(6), pp. 1118-1134. doi: 10.1111/jdv.18931

Antiga E, Bech R, Maglie R, Genovese G, Borradori L, Bockle B, and sur.. S2k guidelines on the management of paraneoplastic pemphigus/paraneoplastic autoimmune multiorgan syndrome initiated by the European Academy of Dermatology and Venereology (EADV). Journal of the European Academy of Dermatology and Venereology [Internet]. 2023 March 25 [cited 2024 May 28];37(6):1118-1134. doi: 10.1111/jdv.18931

E. Antiga, et al., "S2k guidelines on the management of paraneoplastic pemphigus/paraneoplastic autoimmune multiorgan syndrome initiated by the European Academy of Dermatology and Venereology (EADV)", Journal of the European Academy of Dermatology and Venereology, vol. 37, no. 6, pp. 1118-1134, March 2023. [Online]. Available at: https://urn.nsk.hr/urn:nbn:hr:105:818244. [Accessed: 28 May 2024]

Metadata
Title (english)S2k guidelines on the management of paraneoplastic pemphigus/paraneoplastic autoimmune multiorgan syndrome initiated by the European Academy of Dermatology and Venereology (EADV)
AuthorEmiliano Antiga
AuthorRikke Bech
AuthorRoberto Maglie
AuthorGiovanni Genovese
AuthorLuca Borradori
AuthorBarbara Bockle
AuthorMarzia Caproni
AuthorFrédéric Caux
AuthorNisha Suyien Chandran
AuthorAlberto Corrà
AuthorFrancesco D'Amore
AuthorMaryam Daneshpazhooh
AuthorDipankar De
AuthorDario Didona
AuthorMarian Dmochowski
AuthorKossara Drenovska
AuthorJan Ehrchen
AuthorClaudio Feliciani
AuthorMatthias Goebeler
AuthorRichard Groves
AuthorClaudia Günther
AuthorSanjeev Handa
AuthorSilke C. Hofmann
AuthorBarbara Horvath
AuthorDimitrios Ioannidis
AuthorHana Jedlickova
AuthorCezary Kowalewski
AuthorKhalaf Kridin
AuthorPascal Joly
AuthorYen Loo Lim
AuthorBranka Marinović
AuthorEmanual Maverakis
AuthorJoost Meijer
AuthorAikaterini Patsatsi
AuthorCarlo Pincelli
AuthorCatherine Prost
AuthorJane Setterfield
AuthorEli Sprecher
AuthorDusan Skiljevic
AuthorKaisa Tasanen
AuthorSoner Uzun
AuthorNina Van Beek
AuthorSnejina Vassileva
AuthorArtem Vorobyev
AuthorIgor Vujic
AuthorGang Wang
AuthorMingyue Wang
AuthorKatarzyna Wozniak
AuthorSavas Yayli
AuthorGiovanna Zambruno
AuthorTakashi Hashimoto
AuthorEnno Schmidt
AuthorJosé Manuel Mascarò
AuthorAngelo Valerio Marzano
Author's institutionUniversity of Zagreb
School of Medicine
(Department of Dermatology)
Scientific / art field,
discipline and subdiscipline		BIOMEDICINE AND HEALTHCARE
Clinical Medical Sciences
Dermatovenerology
Abstract (english)
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.
Keywords (english)
Languageenglish
Publication typeProfessional paper - Professional paper
Publication statusPublished
Peer reviewPeer review
Publication versionPublished version
Journal titleJournal of the European Academy of Dermatology and Venereology
Numberingvol. 37, no. 6, pp. 1118-1134
p-ISSN0926-9959
e-ISSN1468-3083
DOIhttps://doi.org/10.1111/jdv.18931
URN:NBNurn:nbn:hr:105:818244
Publication2023-03-25
Document URLhttps://onlinelibrary.wiley.com/doi/10.1111/jdv.18931
Type of resourceText
Access conditionsOpen access
Terms of useLicence Attribution-NonCommercial 4.0 International (CC BY-NC 4.0) icon
Created on2024-01-09 08:43:32