Updated S2K guidelines on the management of pemphigus vulgaris and foliaceus initiated by the european academy of dermatology and venereology (EADV)

被引：221

作者：

Joly, P. ^{[1
,2
]}

Horwath, B. ^{[3
]}

Patsatsi, A. ^{[4
]}

Uzun, S. ^{[5
]}

Bech, R. ^{[6
]}

Beissert, S. ^{[7
]}

Bergman, R. ^{[8
]}

Bernard, P. ^{[9
]}

Borradori, L. ^{[10
]}

Caproni, M. ^{[11
]}

Caux, F. ^{[12
,13
]}

Cianchini, G. ^{[14
]}

Daneshpazhooh, M. ^{[15
]}

De, D. ^{[16
]}

Dmochowski, M. ^{[17
]}

Drenovska, K. ^{[18
]}

Ehrchen, J. ^{[19
]}

Feliciani, C. ^{[20
]}

Goebeler, M. ^{[21
]}

Groves, R. ^{[22
]}

Guenther, C. ^{[23
]}

Hofmann, S. ^{[24
]}

Ioannides, D. ^{[25
]}

Kowalewski, C. ^{[26
]}

Ludwig, R. ^{[23
]}

Lim, Y. L. ^{[27
]}

Marinovic, B. ^{[28
]}

Marzano, A., V ^{[29
,30
]}

Mascaro, J. M. Jr Jr ^{[31
]}

Mimouni, D. ^{[32
,33
]}

Murrell, D. F. ^{[34
]}

Pincelli, C. ^{[35
]}

Squarcioni, C. P. ^{[12
,13
]}

Sardy, M. ^{[36
]}

Setterfield, J. ^{[37
,38
]}

Sprecher, E. ^{[39
,40
]}

Vassileva, S. ^{[18
]}

Wozniak, K. ^{[26
]}

Yayli, S. ^{[41
]}

Zambruno, G. ^{[42
]}

Zillikens, D. ^{[43
,44
]}

Hertl, M. ^{[45
]}

Schmidt, E. ^{[43
,44
]}

机构：

[1] Normandie Univ, Rouen Univ Hosp, Dept Dermatol, Rouen, France

[2] Normandie Univ, INSERM U905, Ctr Reference Malad Bulleuses Autoimmunes, Rouen, France

[3] Univ Groningen, Univ Med Ctr Groningen, Ctr Blistering Dis, Dept Dermatol, Groningen, Netherlands

[4] Ristotle Univ, Papageorgiou Gen Hosp, Dermatol Dept 2, Autoimmune Bullous Dis Unit,Sch Med, Thessaloniki, Greece

[5] Akdeniz Univ, Dept Dermatol, Fac Med, Antalya, Turkey

[6] Aarhus Univ Hosp, Dept Dermatol, Aarhus, Denmark

[7] Univ Hosp Carl Gustav Carus, Dept Dermatol, Dresden, Germany

[8] Fac Med, Dept Dermatol, Rambam Hlth Care Campus & Ruth & Bruce Rappaport, Haifa, Israel

[9] Univ Reims, Reims, France

[10] Univ Bern, Univ Hosp Bern, Dept Dermatol, Bern, Switzerland

[11] Univ Florence, USL Toscana Ctr, Dept Hlth Sci, Rare Dis Unit,Sect Dermatol, Florence, Italy

[12] Univ Paris 13, Avicenne Hosp, AP HP, Dept Dermatol, Bobigny, France

[13] Univ Paris 13, Avicenne Hosp, AP HP, Referral Ctr Autoimmune Bullous Dis MALIBUL, Bobigny, France

[14] Cristo Re Hosp, Dept Dermatol, Rome, Italy

[15] Univ Tehran Med Sci, Razi Hosp, Autoimmune Bullous Dis Res Ctr, Dept Dermatol, Tehran, Iran

[16] Postgrad Inst Med Educ & Res, Dept Dermatol, Chandigarh, India

[17] Poznan Univ Med Sci, Dept Dermatol, Autoimmune Blistering Dermatoses Sect, Poznan, Poland

[18] Med Univ, Dept Dermatol, Sofia, Bulgaria

[19] Univ Munster, Dept Dermatol, Munster, Germany

[20] Azienda Osped Univ, Univ Parma, Dermatol Unit, Parma, Italy

[21] Univ Hosp Wurzburg, Dept Dermatol Venereol & Allergol, Wurzburg, Germany

[22] St Johns Inst Dermatol, London, England

[23] Tech Univ Dresden, Univ Hosp, Dept Dermatol, Dresden, Germany

[24] Univ Witten, Helios Univ Hosp Wuppertal, Dept Dermatol Allergy & Dermatosurg, Herdecke, Germany

[25] Aristotle Univ Thessaloniki, Hosp Skin & Venereal Dis, Dept Dermatol Venereol 1, Med Sch, Thessaloniki, Greece

[26] Med Univ Warsaw, Dept Dermatol & Immunodermatol, Warsaw, Poland

[27] Natl Skin Ctr, Singapore, Singapore

[28] Univ Zagreb, Univ Hosp Ctr Zagreb, Sch Med, Dept Dermatol & Venereol, Zagreb, Croatia

[29] Fdn IRCCS Ca Granda Osped Maggiore Policlin, Dermatol Unit, Milan, Italy

[30] Univ Milan, Dept Physiopathol & Transplantat, Milan, Italy

[31] Univ Barcelona, Hosp Clin Barcelona, Dept Dermatol, Barcelona, Spain

[32] Rabin Med Ctr, Dept Dermatol, Petha Tikwa, Israel

[33] Tel Aviv Univ, Sackler Sch Med, Tel Aviv, Israel

[34] Univ NSW, Dept Dermatol, St George Hosp, Sydney, NSW, Australia

[35] Univ Modena & Reggio Emilia, Inst Dermatol, DermoLab, Modena, Italy

[36] Semmelweis Univ, Dept Dermatol Venereol & Dermatooncol, Budapest, Hungary

[37] St Johns Inst Dermatol, Dept Oral Med, London, England

[38] Guys & St Thomas NHS Fdn Trust, London, England

[39] Tel Aviv Sourasky Med Ctr, Div Dermatol, Tel Aviv, Israel

[40] Tel Aviv Univ, Sackler Fac Med, Dept Human Mol Genet & Biochem, Tel Aviv, Israel

[41] Karadeniz Tech Univ, Dept Dermatol, Fac Med, Trabzon, Turkey

[42] IRCCS, Genet & Rare Dis Res Div, Bambino Gesu Childrens Hosp, Rome, Italy

[43] Univ Lubeck, Dept Dermatol, Lubeck, Germany

[44] Univ Lubeck, Lubeck Inst Expt Dermatol LIED, Lubeck, Germany

[45] Univ Marburg, Dept Dermatol, Marburg, Germany

来源：

JOURNAL OF THE EUROPEAN ACADEMY OF DERMATOLOGY AND VENEREOLOGY | 2020年 / 34卷 / 09期

关键词：

AUTOIMMUNE BULLOUS DISEASE; MYCOPHENOLATE-MOFETIL; DOUBLE-BLIND; DESMOGLEIN; RITUXIMAB; THERAPY; MULTICENTER; IMMUNOADSORPTION; AZATHIOPRINE; COMBINATION;

D O I：

10.1111/jdv.16752

中图分类号：

R75 [皮肤病学与性病学];

学科分类号：

100206 ;

摘要：

Background Pemphigus encompasses a group of life-threatening autoimmune bullous diseases characterized by blisters and erosions of the mucous membranes and skin. Before the era of immunosuppressive treatment, pemphigus was almost always fatal. Due to its rarity, only few randomized controlled therapeutic trials are available. Recently, rituximab has been approved as first-line treatment for moderate and severe pemphigus vulgaris in Europe and the United States. Objectives TheAutoimmune blistering diseasesTask Force of the European Academy of Dermatology and Venereology (EADV) has initiated a throughout update of the guideline for the management of patients with pemphigus. Results The guidelines for the management of pemphigus were updated, and the degree of consent among all task force members was included. The final version of the guideline was consented by the European Dermatology Forum (EDF) and several patient organizations.

引用

页码：1900 / 1913

页数：14

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