Updated S2 K guidelines for the management of bullous pemphigoid initiated by the European Academy of Dermatology and Venereology (EADV)

L. Borradori; N. Van Beek; C. Feliciani; B. Tedbirt; E. Antiga; R. Bergman; B. C. Böckle; M. Caproni; F. Caux; N. S. Chandran; G. Cianchini; M. Daneshpazhooh; D. De; D. Didona; G. M. Di Zenzo; M. Dmochowski; K. Drenovska; J. Ehrchen; M. Goebeler; R. Groves; C. Günther; B. Horvath; M. Hertl; S. Hofmann; D. Ioannides; B. Itzlinger-Monshi; J. Jedličková; C. Kowalewski; K. Kridin; Y. L. Lim; B. Marinovic; A. V. Marzano; J. M. Mascaro; J. M. Meijer; D. Murrell; K. Patsatsi; C. Pincelli; C. Prost; K. Rappersberger; M. Sárdy; J. Setterfield; M. Shahid; E. Sprecher; K. Tasanen; S. Uzun; S. Vassileva; K. Vestergaard; A. Vorobyev; I. Vujic; G. Wang; K. Wozniak; S. Yayli; G. Zambruno; D. Zillikens; E. Schmidt; P. Joly

doi:10.1111/jdv.18220

Updated S2 K guidelines for the management of bullous pemphigoid initiated by the European Academy of Dermatology and Venereology (EADV)

L. Borradori^*, N. Van Beek, C. Feliciani, B. Tedbirt, E. Antiga, R. Bergman, B. C. Böckle, M. Caproni, F. Caux, N. S. Chandran, G. Cianchini, M. Daneshpazhooh, D. De, D. Didona, G. M. Di Zenzo, M. Dmochowski, K. Drenovska, J. Ehrchen, M. Goebeler, R. GrovesC. Günther, B. Horvath, M. Hertl, S. Hofmann, D. Ioannides, B. Itzlinger-Monshi, J. Jedličková, C. Kowalewski, K. Kridin, Y. L. Lim, B. Marinovic, A. V. Marzano, J. M. Mascaro, J. M. Meijer, D. Murrell, K. Patsatsi, C. Pincelli, C. Prost, K. Rappersberger, M. Sárdy, J. Setterfield, M. Shahid, E. Sprecher, K. Tasanen, S. Uzun, S. Vassileva, K. Vestergaard, A. Vorobyev, I. Vujic, G. Wang, K. Wozniak, S. Yayli, G. Zambruno, D. Zillikens, E. Schmidt, P. Joly^*

^*Corresponding author for this work

52 Citations (Scopus)

Abstract

Background: Bullous pemphigoid (BP) is the most common autoimmune subepidermal blistering disease of the skin and mucous membranes. This disease typically affects the elderly and presents with itch and localized or, most frequently, generalized bullous lesions. A subset of patients only develops excoriations, prurigo-like lesions, and eczematous and/or urticarial erythematous lesions. The disease, which is significantly associated with neurological disorders, has high morbidity and severely impacts the quality of life. Objectives and methodology: The Autoimmune blistering diseases Task Force of the European Academy of Dermatology and Venereology sought to update the guidelines for the management of BP based on new clinical information, and new evidence on diagnostic tools and interventions. The recommendations are either evidence-based or rely on expert opinion. The degree of consent among all task force members was included. Results: Treatment depends on the severity of BP and patients' comorbidities. High-potency topical corticosteroids are recommended as the mainstay of treatment whenever possible. Oral prednisone at a dose of 0.5 mg/kg/day is a recommended alternative. In case of contraindications or resistance to corticosteroids, immunosuppressive therapies, such as methotrexate, azathioprine, mycophenolate mofetil or mycophenolate acid, may be recommended. The use of doxycycline and dapsone is controversial. They may be recommended, in particular, in patients with contraindications to oral corticosteroids. B-cell-depleting therapy and intravenous immunoglobulins may be considered in treatment-resistant cases. Omalizumab and dupilumab have recently shown promising results. The final version of the guideline was consented to by several patient organizations. Conclusions: The guidelines for the management of BP were updated. They summarize evidence- and expert-based recommendations useful in clinical practice.

Original language	English
Journal	Journal of the European Academy of Dermatology and Venereology
Volume	36
Issue number	10
Pages (from-to)	1689-1704
Number of pages	16
ISSN	0926-9959
DOIs	https://doi.org/10.1111/jdv.18220
Publication status	Published - 10.2022

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

2.21-05 Immunology
2.22-19 Dermatology

UN SDGs

This output contributes to the following UN Sustainable Development Goals (SDGs)

Access to Document

10.1111/jdv.18220

Cite this

Borradori, L., Van Beek, N., Feliciani, C., Tedbirt, B., Antiga, E., Bergman, R., Böckle, B. C., Caproni, M., Caux, F., Chandran, N. S., Cianchini, G., Daneshpazhooh, M., De, D., Didona, D., Di Zenzo, G. M., Dmochowski, M., Drenovska, K., Ehrchen, J., Goebeler, M., ... Joly, P. (2022). Updated S2 K guidelines for the management of bullous pemphigoid initiated by the European Academy of Dermatology and Venereology (EADV). Journal of the European Academy of Dermatology and Venereology, 36(10), 1689-1704. https://doi.org/10.1111/jdv.18220

@article{9a5503d6805846308aa75ac370ff25e6,

title = "Updated S2 K guidelines for the management of bullous pemphigoid initiated by the European Academy of Dermatology and Venereology (EADV)",

abstract = "Background: Bullous pemphigoid (BP) is the most common autoimmune subepidermal blistering disease of the skin and mucous membranes. This disease typically affects the elderly and presents with itch and localized or, most frequently, generalized bullous lesions. A subset of patients only develops excoriations, prurigo-like lesions, and eczematous and/or urticarial erythematous lesions. The disease, which is significantly associated with neurological disorders, has high morbidity and severely impacts the quality of life. Objectives and methodology: The Autoimmune blistering diseases Task Force of the European Academy of Dermatology and Venereology sought to update the guidelines for the management of BP based on new clinical information, and new evidence on diagnostic tools and interventions. The recommendations are either evidence-based or rely on expert opinion. The degree of consent among all task force members was included. Results: Treatment depends on the severity of BP and patients' comorbidities. High-potency topical corticosteroids are recommended as the mainstay of treatment whenever possible. Oral prednisone at a dose of 0.5 mg/kg/day is a recommended alternative. In case of contraindications or resistance to corticosteroids, immunosuppressive therapies, such as methotrexate, azathioprine, mycophenolate mofetil or mycophenolate acid, may be recommended. The use of doxycycline and dapsone is controversial. They may be recommended, in particular, in patients with contraindications to oral corticosteroids. B-cell-depleting therapy and intravenous immunoglobulins may be considered in treatment-resistant cases. Omalizumab and dupilumab have recently shown promising results. The final version of the guideline was consented to by several patient organizations. Conclusions: The guidelines for the management of BP were updated. They summarize evidence- and expert-based recommendations useful in clinical practice.",

author = "L. Borradori and {Van Beek}, N. and C. Feliciani and B. Tedbirt and E. Antiga and R. Bergman and B{\"o}ckle, {B. C.} and M. Caproni and F. Caux and Chandran, {N. S.} and G. Cianchini and M. Daneshpazhooh and D. De and D. Didona and {Di Zenzo}, {G. M.} and M. Dmochowski and K. Drenovska and J. Ehrchen and M. Goebeler and R. Groves and C. G{\"u}nther and B. Horvath and M. Hertl and S. Hofmann and D. Ioannides and B. Itzlinger-Monshi and J. Jedli{\v c}kov{\'a} and C. Kowalewski and K. Kridin and Lim, {Y. L.} and B. Marinovic and Marzano, {A. V.} and Mascaro, {J. M.} and Meijer, {J. M.} and D. Murrell and K. Patsatsi and C. Pincelli and C. Prost and K. Rappersberger and M. S{\'a}rdy and J. Setterfield and M. Shahid and E. Sprecher and K. Tasanen and S. Uzun and S. Vassileva and K. Vestergaard and A. Vorobyev and I. Vujic and G. Wang and K. Wozniak and S. Yayli and G. Zambruno and D. Zillikens and E. Schmidt and P. Joly",

note = "Publisher Copyright: {\textcopyright} 2022 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.",

year = "2022",

month = oct,

doi = "10.1111/jdv.18220",

language = "English",

volume = "36",

pages = "1689--1704",

journal = "Journal of the European Academy of Dermatology and Venereology",

issn = "0926-9959",

number = "10",

}

Borradori, L, Van Beek, N, Feliciani, C, Tedbirt, B, Antiga, E, Bergman, R, Böckle, BC, Caproni, M, Caux, F, Chandran, NS, Cianchini, G, Daneshpazhooh, M, De, D, Didona, D, Di Zenzo, GM, Dmochowski, M, Drenovska, K, Ehrchen, J, Goebeler, M, Groves, R, Günther, C, Horvath, B, Hertl, M, Hofmann, S, Ioannides, D, Itzlinger-Monshi, B, Jedličková, J, Kowalewski, C, Kridin, K, Lim, YL, Marinovic, B, Marzano, AV, Mascaro, JM, Meijer, JM, Murrell, D, Patsatsi, K, Pincelli, C, Prost, C, Rappersberger, K, Sárdy, M, Setterfield, J, Shahid, M, Sprecher, E, Tasanen, K, Uzun, S, Vassileva, S, Vestergaard, K, Vorobyev, A, Vujic, I, Wang, G, Wozniak, K, Yayli, S, Zambruno, G, Zillikens, D, Schmidt, E & Joly, P 2022, 'Updated S2 K guidelines for the management of bullous pemphigoid initiated by the European Academy of Dermatology and Venereology (EADV)', Journal of the European Academy of Dermatology and Venereology, vol. 36, no. 10, pp. 1689-1704. https://doi.org/10.1111/jdv.18220

Updated S2 K guidelines for the management of bullous pemphigoid initiated by the European Academy of Dermatology and Venereology (EADV). / Borradori, L.; Van Beek, N.; Feliciani, C. et al.
In: Journal of the European Academy of Dermatology and Venereology, Vol. 36, No. 10, 10.2022, p. 1689-1704.

Research output: Journal Articles › Journal articles › Research › peer-review

TY - JOUR

T1 - Updated S2 K guidelines for the management of bullous pemphigoid initiated by the European Academy of Dermatology and Venereology (EADV)

AU - Borradori, L.

AU - Van Beek, N.

AU - Feliciani, C.

AU - Tedbirt, B.

AU - Antiga, E.

AU - Bergman, R.

AU - Böckle, B. C.

AU - Caproni, M.

AU - Caux, F.

AU - Chandran, N. S.

AU - Cianchini, G.

AU - Daneshpazhooh, M.

AU - De, D.

AU - Didona, D.

AU - Di Zenzo, G. M.

AU - Dmochowski, M.

AU - Drenovska, K.

AU - Ehrchen, J.

AU - Goebeler, M.

AU - Groves, R.

AU - Günther, C.

AU - Horvath, B.

AU - Hertl, M.

AU - Hofmann, S.

AU - Ioannides, D.

AU - Itzlinger-Monshi, B.

AU - Jedličková, J.

AU - Kowalewski, C.

AU - Kridin, K.

AU - Lim, Y. L.

AU - Marinovic, B.

AU - Marzano, A. V.

AU - Mascaro, J. M.

AU - Meijer, J. M.

AU - Murrell, D.

AU - Patsatsi, K.

AU - Pincelli, C.

AU - Prost, C.

AU - Rappersberger, K.

AU - Sárdy, M.

AU - Setterfield, J.

AU - Shahid, M.

AU - Sprecher, E.

AU - Tasanen, K.

AU - Uzun, S.

AU - Vassileva, S.

AU - Vestergaard, K.

AU - Vorobyev, A.

AU - Vujic, I.

AU - Wang, G.

AU - Wozniak, K.

AU - Yayli, S.

AU - Zambruno, G.

AU - Zillikens, D.

AU - Schmidt, E.

AU - Joly, P.

N1 - Publisher Copyright: © 2022 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.

PY - 2022/10

Y1 - 2022/10

N2 - Background: Bullous pemphigoid (BP) is the most common autoimmune subepidermal blistering disease of the skin and mucous membranes. This disease typically affects the elderly and presents with itch and localized or, most frequently, generalized bullous lesions. A subset of patients only develops excoriations, prurigo-like lesions, and eczematous and/or urticarial erythematous lesions. The disease, which is significantly associated with neurological disorders, has high morbidity and severely impacts the quality of life. Objectives and methodology: The Autoimmune blistering diseases Task Force of the European Academy of Dermatology and Venereology sought to update the guidelines for the management of BP based on new clinical information, and new evidence on diagnostic tools and interventions. The recommendations are either evidence-based or rely on expert opinion. The degree of consent among all task force members was included. Results: Treatment depends on the severity of BP and patients' comorbidities. High-potency topical corticosteroids are recommended as the mainstay of treatment whenever possible. Oral prednisone at a dose of 0.5 mg/kg/day is a recommended alternative. In case of contraindications or resistance to corticosteroids, immunosuppressive therapies, such as methotrexate, azathioprine, mycophenolate mofetil or mycophenolate acid, may be recommended. The use of doxycycline and dapsone is controversial. They may be recommended, in particular, in patients with contraindications to oral corticosteroids. B-cell-depleting therapy and intravenous immunoglobulins may be considered in treatment-resistant cases. Omalizumab and dupilumab have recently shown promising results. The final version of the guideline was consented to by several patient organizations. Conclusions: The guidelines for the management of BP were updated. They summarize evidence- and expert-based recommendations useful in clinical practice.

AB - Background: Bullous pemphigoid (BP) is the most common autoimmune subepidermal blistering disease of the skin and mucous membranes. This disease typically affects the elderly and presents with itch and localized or, most frequently, generalized bullous lesions. A subset of patients only develops excoriations, prurigo-like lesions, and eczematous and/or urticarial erythematous lesions. The disease, which is significantly associated with neurological disorders, has high morbidity and severely impacts the quality of life. Objectives and methodology: The Autoimmune blistering diseases Task Force of the European Academy of Dermatology and Venereology sought to update the guidelines for the management of BP based on new clinical information, and new evidence on diagnostic tools and interventions. The recommendations are either evidence-based or rely on expert opinion. The degree of consent among all task force members was included. Results: Treatment depends on the severity of BP and patients' comorbidities. High-potency topical corticosteroids are recommended as the mainstay of treatment whenever possible. Oral prednisone at a dose of 0.5 mg/kg/day is a recommended alternative. In case of contraindications or resistance to corticosteroids, immunosuppressive therapies, such as methotrexate, azathioprine, mycophenolate mofetil or mycophenolate acid, may be recommended. The use of doxycycline and dapsone is controversial. They may be recommended, in particular, in patients with contraindications to oral corticosteroids. B-cell-depleting therapy and intravenous immunoglobulins may be considered in treatment-resistant cases. Omalizumab and dupilumab have recently shown promising results. The final version of the guideline was consented to by several patient organizations. Conclusions: The guidelines for the management of BP were updated. They summarize evidence- and expert-based recommendations useful in clinical practice.

UR - http://www.scopus.com/inward/record.url?scp=85133041862&partnerID=8YFLogxK

U2 - 10.1111/jdv.18220

DO - 10.1111/jdv.18220

M3 - Journal articles

C2 - 35766904

AN - SCOPUS:85133041862

SN - 0926-9959

VL - 36

SP - 1689

EP - 1704

JO - Journal of the European Academy of Dermatology and Venereology

JF - Journal of the European Academy of Dermatology and Venereology

IS - 10

ER -

Updated S2 K guidelines for the management of bullous pemphigoid initiated by the European Academy of Dermatology and Venereology (EADV)

Abstract

Research Areas and Centers

DFG Research Classification Scheme

UN SDGs

Access to Document

Other files and links

Fingerprint

CRC 1526, PANTAU: Pathomechanisms of Antibody-mediated Autoimmunity

Cite this

Updated S2 K guidelines for the management of bullous pemphigoid initiated by the European Academy of Dermatology and Venereology (EADV)

Abstract

Research Areas and Centers

DFG Research Classification Scheme

UN SDGs

Access to Document

Other files and links

Fingerprint

Projects

CRC 1526, PANTAU: Pathomechanisms of Antibody-mediated Autoimmunity

Cite this