S2k Guidelines on the Management of Paraneoplastic Pemphigus/Paraneoplastic Autoimmune Multiorgan Syndrome Initiated by the European Academy of Dermatology and Venereology (Eadv)

S2k Guidelines on the Management of Paraneoplastic Pemphigus/Paraneoplastic Autoimmune Multiorgan Syndrome Initiated by the European Academy of Dermatology and Venereology (Eadv) Publisher Pubmed

Antiga E¹ ; Bech R² ; Maglie R¹ ; Genovese G³ ; Borradori L⁴ ; Bockle B⁵ ; Caproni M^{1, 6} ; Caux F⁷ ; Chandran NS^{8, 9} ; Corra A¹ ; Damore F¹⁰ ; Daneshpazhooh M¹¹ ; De D¹² ; Didona D¹³ Show All Authors

Antiga E¹
Bech R²
Maglie R¹
Genovese G³
Borradori L⁴
Bockle B⁵
Caproni M^{1, 6}
Caux F⁷
Chandran NS^{8, 9}
Corra A¹
Damore F¹⁰
Daneshpazhooh M¹¹
De D¹²
Didona D¹³
Dmochowski M¹⁴
Drenovska K¹⁵
Ehrchen J¹⁶
Feliciani C¹⁷
Goebeler M¹⁸
Groves R¹⁹
Gunther C²⁰
Handa S²¹
Hofmann SC²²
Horvath B²³
Ioannidis D²⁴
Jedlickova H²⁵
Kowalewski C²⁶
Kridin K^{27, 28}
Joly P²⁹
Lim YL^{30, 31}
Marinovic B³²
Maverakis E³³
Meijer J²³
Patsatsi A³⁴
Pincelli C³⁵
Prost C³⁶
Setterfield J³⁷
Sprecher E^{38, 39}
Skiljevic D^{40, 41}
Tasanen K⁴²
Uzun S⁴³
Vanbeek N⁴⁴
Vassileva S¹⁵
Vorobyev A⁴⁴
Vujic I^{45, 46}
Wang G⁴⁷
Wang M^{48, 49}
Wozniak K⁵⁰
Yayli S⁵¹
Zambruno G⁵²
Hashimoto T⁵³
Schmidt E^{44, 54}
Marzano AV⁵⁶

Source: Journal of the European Academy of Dermatology and Venereology Published:2023

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. © 2023 The Authors. Journal of the European Academy of Dermatology and Venereology published by John Wiley & Sons Ltd on behalf of European Academy of Dermatology and Venereology.

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Style	Citing Format
MLA	Antiga E, 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.
APA	Antiga E, Bech R, Maglie R, Genovese G, Borradori L, Bockle B, Caproni M, Caux F, Chandran NS, Corra A, Damore F, Daneshpazhooh M, De D, Didona D, Dmochowski M, Drenovska K, Ehrchen J, Feliciani C, Goebeler M, ... Marzano AV (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.
Chicago	Antiga E, Bech R, Maglie R, Genovese G, Borradori L, Bockle B, Caproni M, 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.
Harvard	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.
Vancouver	Antiga E, Bech R, Maglie R, Genovese G, Borradori L, Bockle B, 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. 2023;37(6):1118-1134.
BibTex	@article{ author = {Antiga E and Bech R and Maglie R and Genovese G and Borradori L and Bockle B and Caproni M and Caux F and Chandran NS and Corra A and Damore F and Daneshpazhooh M and De D and Didona D and Dmochowski M and Drenovska K and Ehrchen J and Feliciani C and Goebeler M and Groves R and Gunther C and Handa S and Hofmann SC and Horvath B and Ioannidis D and Jedlickova H and Kowalewski C and Kridin K and Joly P and Lim YL and Marinovic B and Maverakis E and Meijer J and Patsatsi A and Pincelli C and Prost C and Setterfield J and Sprecher E and Skiljevic D and Tasanen K and Uzun S and Vanbeek N and Vassileva S and Vorobyev A and Vujic I and Wang G and Wang M and Wozniak K and Yayli S and Zambruno G and Hashimoto T and Schmidt E and Marzano AV}, title = {S2k Guidelines on the Management of Paraneoplastic Pemphigus/Paraneoplastic Autoimmune Multiorgan Syndrome Initiated by the European Academy of Dermatology and Venereology (Eadv)}, journal = {Journal of the European Academy of Dermatology and Venereology}, volume = {37}, number = {6}, pages = {1118-1134}, year = {2023} }
RIS	TY - JOUR AU - Antiga E AU - Bech R AU - Maglie R AU - Genovese G AU - Borradori L AU - Bockle B AU - Caproni M AU - Caux F AU - Chandran NS AU - Corra A AU - Damore F AU - Daneshpazhooh M AU - De D AU - Didona D AU - Dmochowski M AU - Drenovska K AU - Ehrchen J AU - Feliciani C AU - Goebeler M AU - Groves R AU - Gunther C AU - Handa S AU - Hofmann SC AU - Horvath B AU - Ioannidis D AU - Jedlickova H AU - Kowalewski C AU - Kridin K AU - Joly P AU - Lim YL AU - Marinovic B AU - Maverakis E AU - Meijer J AU - Patsatsi A AU - Pincelli C AU - Prost C AU - Setterfield J AU - Sprecher E AU - Skiljevic D AU - Tasanen K AU - Uzun S AU - Vanbeek N AU - Vassileva S AU - Vorobyev A AU - Vujic I AU - Wang G AU - Wang M AU - Wozniak K AU - Yayli S AU - Zambruno G AU - Hashimoto T AU - Schmidt E AU - Marzano AV TI - S2k Guidelines on the Management of Paraneoplastic Pemphigus/Paraneoplastic Autoimmune Multiorgan Syndrome Initiated by the European Academy of Dermatology and Venereology (Eadv) JO - Journal of the European Academy of Dermatology and Venereology VL - 37 IS - 6 SP - 1118 EP - 1134 PY - 2023 ER -

Style

Citing Format

MLA

Antiga E, 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.

APA

Antiga E, Bech R, Maglie R, Genovese G, Borradori L, Bockle B, Caproni M, Caux F, Chandran NS, Corra A, Damore F, Daneshpazhooh M, De D, Didona D, Dmochowski M, Drenovska K, Ehrchen J, Feliciani C, Goebeler M, ... Marzano AV (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.

Chicago

Antiga E, Bech R, Maglie R, Genovese G, Borradori L, Bockle B, Caproni M, 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.

Harvard

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.

Vancouver

Antiga E, Bech R, Maglie R, Genovese G, Borradori L, Bockle B, 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. 2023;37(6):1118-1134.

BibTex

@article{ author = {Antiga E and Bech R and Maglie R and Genovese G and Borradori L and Bockle B and Caproni M and Caux F and Chandran NS and Corra A and Damore F and Daneshpazhooh M and De D and Didona D and Dmochowski M and Drenovska K and Ehrchen J and Feliciani C and Goebeler M and Groves R and Gunther C and Handa S and Hofmann SC and Horvath B and Ioannidis D and Jedlickova H and Kowalewski C and Kridin K and Joly P and Lim YL and Marinovic B and Maverakis E and Meijer J and Patsatsi A and Pincelli C and Prost C and Setterfield J and Sprecher E and Skiljevic D and Tasanen K and Uzun S and Vanbeek N and Vassileva S and Vorobyev A and Vujic I and Wang G and Wang M and Wozniak K and Yayli S and Zambruno G and Hashimoto T and Schmidt E and Marzano AV}, title = {S2k Guidelines on the Management of Paraneoplastic Pemphigus/Paraneoplastic Autoimmune Multiorgan Syndrome Initiated by the European Academy of Dermatology and Venereology (Eadv)}, journal = {Journal of the European Academy of Dermatology and Venereology}, volume = {37}, number = {6}, pages = {1118-1134}, year = {2023} }

RIS

TY - JOUR
AU - Antiga E
AU - Bech R
AU - Maglie R
AU - Genovese G
AU - Borradori L
AU - Bockle B
AU - Caproni M
AU - Caux F
AU - Chandran NS
AU - Corra A
AU - Damore F
AU - Daneshpazhooh M
AU - De D
AU - Didona D
AU - Dmochowski M
AU - Drenovska K
AU - Ehrchen J
AU - Feliciani C
AU - Goebeler M
AU - Groves R
AU - Gunther C
AU - Handa S
AU - Hofmann SC
AU - Horvath B
AU - Ioannidis D
AU - Jedlickova H
AU - Kowalewski C
AU - Kridin K
AU - Joly P
AU - Lim YL
AU - Marinovic B
AU - Maverakis E
AU - Meijer J
AU - Patsatsi A
AU - Pincelli C
AU - Prost C
AU - Setterfield J
AU - Sprecher E
AU - Skiljevic D
AU - Tasanen K
AU - Uzun S
AU - Vanbeek N
AU - Vassileva S
AU - Vorobyev A
AU - Vujic I
AU - Wang G
AU - Wang M
AU - Wozniak K
AU - Yayli S
AU - Zambruno G
AU - Hashimoto T
AU - Schmidt E
AU - Marzano AV
TI - S2k Guidelines on the Management of Paraneoplastic Pemphigus/Paraneoplastic Autoimmune Multiorgan Syndrome Initiated by the European Academy of Dermatology and Venereology (Eadv)
JO - Journal of the European Academy of Dermatology and Venereology
VL - 37
IS - 6
SP - 1118
EP - 1134
PY - 2023
ER -

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