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

. 2022 Oct ; 36 (10) : 1689-1704. [epub] 20220629

Jazyk angličtina Země Velká Británie, Anglie Médium print-electronic

Typ dokumentu časopisecké články

Perzistentní odkaz   https://www.medvik.cz/link/pmid35766904

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.

1st Department of Dermatology Venereology Hospital of Skin and Venereal Diseases Aristotle University Medical School Thessaloniki Greece

2nd Department of Dermatology Autoimmune Bullous Diseases Unit Aristotle University School of Medicine Papageorgiou General Hospital Thessaloniki Greece

Abteilung Dermatologie Venerologie und Allergologie Lehrkrankenhaus der Medizinischen Universität Wien Austria

Autoimmune Blistering Dermatoses Section Department of Dermatology Poznan University of Medical Sciences Poznan Poland

Center for Research on Inflammation of the Skin University of Lübeck Lübeck Germany

Department Dermatology and Immunodermatology Medical University of Warsaw Warsaw Poland

Department of Dermatology Aarhus University Hospital Aarhus Denmark

Department of Dermatology Allergy and Dermatosurgery Helios University Hospital Wuppertal University Witten Herdecke Germany

Department of Dermatology and Allergology Ludwig Maximilian University Munich Germany

Department of Dermatology and Allergology Philipps University Marburg Germany

Department of Dermatology and Referral Center for Autoimmune Bullous Diseases Groupe Hospitalier Paris Seine Saint Denis AP HP and University Paris 13 Bobigny France

Department of Dermatology and Venereology Akdeniz University Faculty of Medicine Antalya Turkey

Department of Dermatology and Venereology School of Medicine University Hospital Centre Zagreb University of Zagreb Zagreb Croatia

Department of Dermatology Autoimmune Bullous Diseases Research Center Razi Hospital Tehran University of Medical Sciences Tehran Iran

Department of Dermatology Carl Gustav Carus University Hospital Technische Universität Dresden Dresden Germany

Department of Dermatology Center for Blistering Diseases University Medical Center Groningen University of Groningen Groningen the Netherlands

Department of Dermatology Hospital Clínic de Barcelona Universitat de Barcelona Barcelona Spain

Department of Dermatology Inselspital Bern University Hospital Bern Switzerland

Department of Dermatology Medical University of Sofia Sofia Bulgaria

Department of Dermatology Medical University Sofia Bulgaria

Department of Dermatology Ospedale Classificato Cristo Re Rome Italy

Department of Dermatology Rambam Health Care Campus Haifa Israel

Department of Dermatology Rouen University Hospital Referral Center for Autoimmune Bullous Diseases Referral Center for Autoimmune Bullous Diseases Rouen University Hospital INSERM U1234 Normandie University Rouen France

Department of Dermatology School of Medicine Koç University Istanbul Turkey

Department of Dermatology St George Hospital University of New South Wales Sydney New South Wales Australia

Department of Dermatology the PEDEGO Research Unit University of Oulu and Medical Research Center Oulu Oulu University Hospital Oulu Finland

Department of Dermatology University of Lübeck Lübeck Germany

Department of Dermatology University of Münster Münster Germany

Department of Dermatology Venereology and Allergology Innsbruck Medical University Innsbruck Austria

Department of Dermatology Venereology and Allergology University Hospital Würzburg Würzburg Germany

Department of Dermatology Venereology and Allergy Clinical Center Landstrasse Academic Teaching Hospital of the Medical University of Vienna Vienna Austria

Department of Dermatology Venereology and Dermatooncology Semmelweis University Budapest Hungary

Department of Dermatology Venereology and Leprology Postgraduate Institute of Medical Education and Research Chandigarh India

Department of Dermatology Xijing Hospital 4th Military Medical University Xi'an China

Department of Dermatovenereology Masaryk University University Hospital St Anna Brno

Department of Dermatovenereology University Hospital Brno Brno Czech Republic

Department of Health Sciences Section of Dermatology AUSL Toscana Centro Rare Diseases Unit European Reference Network Skin Member University of Florence Italy

Department of Medicine Yong Loo Lin School of Medicine National University of Singapore Singapore

Department of Oral Medicine St John's Institute of Dermatology Guy's and St Thomas' NHS Foundation Trust London UK

Department of Pathophysiology and Transplantation Università degli Studi di Milano Milan Italy

Dermatology Unit Department of Medicine and Surgery University Hospital University of Parma Italy

Dermatology Unit Fondazione IRCCS Cà Granda Ospedale Maggiore Policlinico Milan Italy

DermoLab Institute of Dermatology University of Modena and Reggio Emilia Modena Italy

Division of Dermatology Tel Aviv Sourasky Medical Center and Department of Human Molecular Genetics and Biochemistry Sackler Faculty of Medicine Tel Aviv University Tel Aviv Israel

Division of Genetics and Molecular Medicine King's College London Guy's Hospital London UK

Genetics and Rare Diseases Research Division Bambino Gesù Children's Hospital IRCCS Rome Italy

Laboratory of Molecular and Cell Biology Istituto Dermopatico dell'Immacolata IDI IRCCS Rome Italy

Lübeck Institute of Experimental Dermatology University of Lübeck Lübeck Germany

Medical Faculty The Sigmund Freud Private University Vienna Austria

National Skin Centre Singapore Singapore

Rappaport Faculty of Medicine Technion Israel Institute of Technology Haifa Israel

Section of Dermatology Department of Health Sciences University of Florence Florence Italy

St John's Institute of Dermatology Viapath Analytics LLP St Thomas' Hospital London UK

Zobrazit více v PubMed

Di Zenzo G, Della Torre R, Zambruno G, Borradori L. Bullous pemphigoid: from the clinic to the bench. Clin Dermatol 2012; 30: 3-16.

Schmidt E, Zillikens D. Pemphigoid diseases. Lancet 2013; 381: 320-332.

Hammers CM, Stanley JR. Recent advances in understanding pemphigus and bullous pemphigoid. J Invest Dermatol 2020; 140: 733-741.

Amber KT, Murrell DF, Schmidt E, Joly P, Borradori L. Autoimmune subepidermal bullous diseases of the skin and mucosae: clinical features, diagnosis, and management. Clin Rev Allergy Immunol 2018; 54: 26-51.

Bernard P, Antonicelli F. Bullous pemphigoid: a review of its diagnosis, associations and treatment. Am J Clin Dermatol 2017; 18: 513-528.

Kridin K, Ludwig RJ. The growing incidence of bullous pemphigoid: overview and potential explanations. Front Med (Lausanne) 2018; 5: 220.

Liu YD, Wang YH, Ye YC, Zhao WL, Li L. Prognostic factors for mortality in patients with bullous pemphigoid: a meta-analysis. Arch Dermatol Res 2017; 309: 335-347.

Venning VA, Taghipour K, Mohd Mustapa MF, Highet AS, Kirtschig G. British Association of Dermatologists' guidelines for the management of bullous pemphigoid 2012. Br J Dermatol 2012; 167: 1200-1214.

Murrell DF, Daniel BS, Joly P et al. Definitions and outcome measures for bullous pemphigoid: recommendations by an international panel of experts. J Am Acad Dermatol 2012; 66: 479-485.

Schulze F, Neumann K, Recke A, Zillikens D, Linder R, Schmidt E. Malignancies in pemphigus and pemphigoid diseases. J Invest Dermatol 2015; 135: 1445-1447.

Bastuji-Garin S, Joly P, Picard-Dahan C et al. Drugs associated with bullous pemphigoid. A case-control study. Arch Dermatol 1996; 132: 272-276.

Kibsgaard L, Rasmussen M, Lamberg A, Deleuran M, Olesen AB, Vestergaard C. Increased frequency of multiple sclerosis among patients with bullous pemphigoid: a population-based cohort study on comorbidities anchored around the diagnosis of bullous pemphigoid. Br J Dermatol 2017; 176: 1486-1491.

Lloyd-Lavery A, Chi CC, Wojnarowska F, Taghipour K. The associations between bullous pemphigoid and drug use: a UK case-control study. JAMA Dermatol 2013; 149: 58-62.

Tasanen K, Varpuluoma O, Nishie W. Dipeptidyl Peptidase-4 inhibitor-associated bullous pemphigoid. Front Immunol 2019; 10: 1238.

Benzaquen M, Borradori L, Berbis P et al. Dipeptidyl peptidase IV inhibitors, a risk factor for bullous pemphigoid: retrospective multicenter case-control study from France and Switzerland. J Am Acad Dermatol 2018; 78: 1090-1096.

Plaquevent M, Tetart F, Fardet L et al. Higher frequency of dipeptidyl Peptidase-4 inhibitor intake in bullous pemphigoid patients than in the French general population. J Invest Dermatol 2019; 139: 835-841.

Sadik CD, Langan EA, Gutzmer R et al. Retrospective analysis of checkpoint inhibitor therapy-associated cases of bullous pemphigoid fom six German dermatology centers. Front Immunol 2021; 11: 588582.

della Torre R, Combescure C, Cortes B et al. Clinical presentation and diagnostic delay in bullous pemphigoid: a prospective nationwide cohort. Br J Dermatol 2012; 167: 1111-1117.

Joly P, Baricault S, Sparsa A et al. Incidence and mortality of bullous pemphigoid in France. J Invest Dermatol 2012; 132: 1998-2004.

Joly P, Courville P, Lok C et al. Clinical criteria for the diagnosis of bullous pemphigoid: a reevaluation according to immunoblot analysis of patient sera. Dermatology 2004; 208: 16-20.

Haefliger S, Sitaru S, Cazzaniga S et al. Diagnostic performance of direct immunofluorescence microscopy studies by biopsy sites in autoimmune subepidermal blistering dermatoses: a prospective study. Br J Dermatol 2020; 183: 970-972.

Kelly SE, Wojnarowska F. The use of chemically split tissue in the detection of circulating anti-basement membrane zone antibodies in bullous pemphigoid and cicatricial pemphigoid. Br J Dermatol 1988; 118: 31-40.

Zillikens D, Mascaro JM, Rose PA et al. A highly sensitive enzyme-linked immunosorbent assay for the detection of circulating anti-BP180 autoantibodies in patients with bullous pemphigoid. J Invest Dermatol 1997; 109: 679-683.

Di Zenzo G, Thoma-Uszynski S, Fontao L et al. Multicenter prospective study of the humoral autoimmune response in bullous pemphigoid. Clin Immunol 2008; 128: 415-426.

Fairley JA, Bream M, Fullenkamp C, Syrbu S, Chen M, Messingham KN. Missing the target: characterization of bullous pemphigoid patients who are negative using the BP180 enzyme-linked immunosorbent assay. J Am Acad Dermatol 2013; 68: 395-403.

van Beek N, Kruger S, Fuhrmann T et al. Multicenter prospective study on multivariant diagnostics of autoimmune bullous dermatoses using the BIOCHIP(TM) technology. J Am Acad Dermatol 2020; 83: 1315-1322.

Machado-Pinto J, McCalmont TH, Golitz LE. Eosinophilic and neutrophilic spongiosis: clues to the diagnosis of immunobullous diseases and other inflammatory disorders. Semin Cutan Med Surg 1996; 15: 308-316.

Terra JB, Meijer JM, Jonkman MF, Diercks GF. The n- vs. u-serration is a learnable criterion to differentiate pemphigoid from epidermolysis bullosa acquisita in direct immunofluorescence serration pattern analysis. Br J Dermatol 2013; 169: 100-105.

Gammon WR, Kowalewski C, Chorzelski TP, Kumar V, Briggaman RA, Beutner EH. Direct immunofluorescence studies of sodium chloride-separated skin in the differential diagnosis of bullous pemphigoid and epidermolysis bullosa acquisita. J Am Acad Dermatol 1990; 22: 664-670.

Chan YC, Sun YJ, Ng PP, Tan SH. Comparison of immunofluorescence microscopy, immunoblotting and enzyme-linked immunosorbent assay methods in the laboratory diagnosis of bullous pemphigoid. Clin Exp Dermatol 2003; 28: 651-656.

Roussel A, Benichou J, Randriamanantany ZA et al. Enzyme-linked immunosorbent assay for the combination of bullous pemphigoid antigens 1 and 2 in the diagnosis of bullous pemphigoid. Arch Dermatol 2011; 147: 293-298.

van Beek N, Dahnrich C, Johannsen N et al. Prospective studies on the routine use of a novel multivariant enzyme-linked immunosorbent assay for the diagnosis of autoimmune bullous diseases. J Am Acad Dermatol 2017; 76: e5.

Horvath ON, Varga R, Kaneda M, Schmidt E, Ruzicka T, Sardy M. Diagnostic performance of the "MESACUP anti-skin profile TEST". Eur J Dermatol 2016; 26: 56-63.

Labib RS, Anhalt GJ, Patel HP, Mutasim DF, Diaz LA. Molecular heterogeneity of the bullous pemphigoid antigens as detected by immunoblotting. J Immunol 1986; 136: 1231-1235.

De Jong MC, Bruins S, Heeres K et al. Bullous pemphigoid and epidermolysis bullosa acquisita. Differentiation by fluorescence overlay antigen mapping. Arch Dermatol 1996; 132: 151-157.

Wozniak K, Kazama T, Kowalewski C. A practical technique for differentiation of subepidermal bullous diseases: localization of in vivo-bound IgG by laser scanning confocal microscopy. Arch Dermatol 2003; 139: 1007-1011.

van Beek N, Rentzsch K, Probst C et al. Serological diagnosis of autoimmune bullous skin diseases: prospective comparison of the BIOCHIP mosaic-based indirect immunofluorescence technique with the conventional multi-step single test strategy. Orphanet J Rare Dis 2012; 7: 49.

Bedane C, Prost C, Bernard P, Catanzano G, Bonnetblanc JM, Dubertret L. Cicatricial pemphigoid antigen differs from bullous pemphigoid antigen by its exclusive extracellular localization: a study by indirect immunoelectron microscopy. J Invest Dermatol 1991; 97: 3-9.

Joly P, Benichou J, Lok C et al. Prediction of survival for patients with bullous pemphigoid: a prospective study. Arch Dermatol 2005; 141: 691-698.

Cortes B, Marazza G, Naldi L, Combescure C, Borradori L. Mortality of bullous pemphigoid in Switzerland: a prospective study. Br J Dermatol 2011; 165: 368-374.

Joly P, Roujeau JC, Benichou J et al. A comparison of two regimens of topical corticosteroids in the treatment of patients with bullous pemphigoid: a multicenter randomized study. J Invest Dermatol 2009; 129: 1681-1687.

Joly P, Roujeau JC, Benichou J et al. A comparison of oral and topical corticosteroids in patients with bullous pemphigoid. N Engl J Med 2002; 346: 321-327.

Bernard P, Reguiai Z, Tancrede-Bohin E et al. Risk factors for relapse in patients with bullous pemphigoid in clinical remission: a multicenter, prospective, cohort study. Arch Dermatol 2009; 145: 537-542.

Kirtschig G, Middleton P, Bennett C, Murrell DF, Wojnarowska F, Khumalo NP. Interventions for bullous pemphigoid. Cochrane Database Syst Rev 2010: 2010: Cd002292.

Daniel BS, Borradori L, Hall RP 3rd, Murrell DF. Evidence-based management of bullous pemphigoid. Dermatol Clin 2011; 29: 613-620.

Singh S. Evidence-based treatments for pemphigus vulgaris, pemphigus foliaceus, and bullous pemphigoid: a systematic review. Indian J Dermatol Venereol Leprol 2011; 77: 456-469.

Hebert V, Bastos S, Drenovska K et al. International multicentre observational study to assess the efficacy and safety of a 0.5 mg/kg/day starting dose of oral corticosteroids to treat bullous pemphigoid. Br J Dermatol 2021; 184: 1106-1112.

Roujeau JC, Guillaume JC, Morel P et al. Plasma exchange in bullous pemphigoid. Lancet 1984; 2: 486-488.

Fivenson DP, Breneman DL, Rosen GB, Hersh CS, Cardone S, Mutasim D. Nicotinamide and tetracycline therapy of bullous pemphigoid. Arch Dermatol 1994; 130: 753-758.

Williams HC, Wojnarowska F, Kirtschig G et al. Doxycycline versus prednisolone as an initial treatment strategy for bullous pemphigoid: a pragmatic, non-inferiority, randomised controlled trial. Lancet 2017; 389: 1630-1638.

Micallef D, Harman KE. Doxycycline in the management of bullous pemphigoid: Real-world data from a specialist Centre. Br J Dermatol 2021; 185(Suppl. 1): 8-9.

Bouscarat F, Chosidow O, Picard-Dahan C et al. Treatment of bullous pemphigoid with dapsone: retrospective study of thirty-six cases. J Am Acad Dermatol 1996; 34: 683-684.

Sticherling M, Franke A, Aberer E et al. An open, multicentre, randomized clinical study in patients with bullous pemphigoid comparing methylprednisolone and azathioprine with methylprednisolone and dapsone. Br J Dermatol 2017; 177: 1299-1305.

Guillaume JC, Vaillant L, Bernard P et al. Controlled trial of azathioprine and plasma exchange in addition to prednisolone in the treatment of bullous pemphigoid. Arch Dermatol 1993; 129: 49-53.

Beissert S, Werfel T, Frieling U et al. A comparison of oral methylprednisolone plus azathioprine or mycophenolate mofetil for the treatment of bullous pemphigoid. Arch Dermatol 2007; 143: 1536-1542.

Bystryn JC. Comparative effectiveness of azathioprine or mycophenolate mofetil as an adjuvant for the treatment of bullous pemphigoid. Arch Dermatol 2008; 144: 946.

Du-Thanh A, Merlet S, Maillard H et al. Combined treatment with low-dose methotrexate and initial short-term superpotent topical steroids in bullous pemphigoid: an open, multicentre, retrospective study. Br J Dermatol 2011; 165: 1337-1343.

Dereure O, Bernard P, Oro S et al. Corticothérapie locale brève+méthotrexate vs corticothérapie locale seule prolongée dans la pemphigoïde bulleuse: essai national multicentrique. Ann Dermatol Venereol 2017; 144: S86.

Barthelemy H, Thivolet J, Cambazard F et al. Cyclosporin in the treatment of bullous pemphigoid: preliminary study. Ann Dermatol Venereol 1986; 113: 309-313.

Schmidt E, Seitz CS, Benoit S, Brocker EB, Goebeler M. Rituximab in autoimmune bullous diseases: mixed responses and adverse effects. Br J Dermatol 2007; 156: 352-356.

Hall RP 3rd, Streilein RD, Hannah DL et al. Association of serum B-cell activating factor level and proportion of memory and transitional B cells with clinical response after rituximab treatment of bullous pemphigoid patients. J Invest Dermatol 2013; 133: 2786-2788.

Joly P. French study group on auto immune bullous skin d, the French network of rare diseases in D. incidence and severity of COVID-19 in patients with autoimmune blistering skin diseases: a nationwide study. J Am Acad Dermatol 2021; 86: 494-497.

Amagai M, Ikeda S, Hashimoto T et al. A randomized double-blind trial of intravenous immunoglobulin for bullous pemphigoid. J Dermatol Sci 2017; 85: 77-84.

Fairley JA, Baum CL, Brandt DS, Messingham KA. Pathogenicity of IgE in autoimmunity: successful treatment of bullous pemphigoid with omalizumab. J Allergy Clin Immunol 2009; 123: 704-705.

Abdat R, Waldman RA, de Bedout V et al. Dupilumab as a novel therapy for bullous pemphigoid: a multicenter case series. J Am Acad Dermatol 2020; 83: 46-52.

Seyed Jafari SM, Feldmeyer L, Bossart S, Simon D, Schlapbach C, Borradori L. Case report: combination of Omalizumab and Dupilumab for recalcitrant bullous pemphigoid. Front Immunol 2020; 11: 611549.

Delaumenie S, Assikar S, Prudhomme R et al. Methotrexate is safe and efficient as long-term treatment for bullous pemphigoid. Eur J Dermatol 2019; 29: 217-218.

Schmidt E, Kraensel R, Goebeler M et al. Treatment of bullous pemphigoid with dapsone, methylprednisolone, and topical clobetasol propionate: a retrospective study of 62 cases. Cutis 2005; 76: 205-209.

Masmoudi W, Vaillant M, Vassileva S et al. International validation of the bullous pemphigoid disease area index severity score and calculation of cut-off values for defining mild, moderate and severe types of bullous pemphigoid. Br J Dermatol 2021; 184: 1106-1112.

Gaitanis G, Alexis I, Pelidou SH et al. High-dose intravenous immunoglobulin in the treatment of adult patients with bullous pemphigoid. Eur J Dermatol 2012; 22: 363-369.

Hubner F, Kasperkiewicz M, Knuth-Rehr D et al. Adjuvant treatment of severe/refractory bullous pemphigoid with protein a immunoadsorption. J Dtsch Dermatol Ges 2018; 16: 1109-1118.

Meyersburg D, Schmidt E, Kasperkiewicz M, Zillikens D. Immunoadsorption in dermatology. Ther Apher Dial 2012; 16: 311-320.

Le Roux-Villet C, Prost-Squarcioni C, Oro S, Roujeau JC, Joly P, Lemercier C. Role of the nurse in care of bullous pemphigoid. Rev Infirm 2010; 160: 38-40.

Egeberg A, Schwarz P, Harslof T et al. Association of potent and very potent topical corticosteroids and the risk of osteoporosis and major osteoporotic fractures. JAMA Dermatol 2021; 157: 275-282.

Grossman JM, Gordon R, Ranganath VK et al. American College of Rheumatology 2010 recommendations for the prevention and treatment of glucocorticoid-induced osteoporosis. Arthritis Care Res (Hoboken) 2010; 62: 1515-1526.

Fichel F, Barbe C, Joly P et al. Clinical and immunologic factors associated with bullous pemphigoid relapse during the first year of treatment: a multicenter, prospective study. JAMA Dermatol 2014; 150: 25-33.

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