GUIDELINES FOR THE SYSTEMIC TREATMENT OF PATIENTS WITH PEMPHIGUS AT THE UNIVERSITY CLINIC FOR DERMATOLOGY – SKOPJE

Authors

  • Julija Mitrova Telenta University Clinic for Dermatology, Faculty of Medicine, Ss. Cyril and Methodius University in Skopje, Republic of North Macedonia
  • Viktor Simeonovski University Clinic for Dermatology, Skopje, North Macedonia, Medical Faculty, University "Ss. Cyril and Methodius" Skopje, Skopje, North Macedonia
  • Bojana Batkoska Shekutkoska University Clinic for Dermatology, Faculty of Medicine, Ss. Cyril and Methodius University in Skopje, Republic of North Macedonia
  • Dejan Filipovikj University Clinic for Dermatology, Faculty of Medicine, Ss. Cyril and Methodius University in Skopje, Republic of North Macedonia
  • Kamelija Doneva Simonov University Clinic for Dermatology, Faculty of Medicine, Ss. Cyril and Methodius University in Skopje, Republic of North Macedonia
  • Tomche Popovski University Clinic for Dermatology, Faculty of Medicine, Ss. Cyril and Methodius University in Skopje, Republic of North Macedonia
  • Anita Najdova University Clinic for Dermatology, Faculty of Medicine, Ss. Cyril and Methodius University in Skopje, Republic of North Macedonia
  • Katerina Damveska University Clinic for Dermatology, Faculty of Medicine, Ss. Cyril and Methodius University in Skopje, Republic of North Macedonia

Keywords:

pemphigus, guidelines

Abstract

Pemphigus encompasses a group of life-threatening autoimmune bullous diseases characterized by blisters and erosions on the mucous membranes and skin, requiring a standardized approach to diagnosis and treatment. According to current recommendations, the treatment of diseases in this group involves the use of systemic corticosteroids in combination with immunosuppressants (azathioprine and mycophenolate mofetil), as well as biological therapy with rituximab, which has already been approved as a first-line treatment for moderate to severe pemphigus vulgaris in Europe and the USA.

These guidelines were developed for the needs of the University Clinic of Dermatology – Skopje, which serves as a referral center for the diagnosis and treatment of pemphigus in the Republic of North Macedonia. They are based on the Updated S2K guidelines on the management of pemphigus vulgaris and foliaceus initiated by the European Academy of Dermatology and Venereology (EADV). The guidelines include general information about the disease, a system for grading disease severity, and recommendations for treatment and monitoring.

References

Joly P, Horwath B, Patsatsi A, et al. Updated S2K guidelines on the management of pemphigus vulgaris and foliaceus initiated by the European Academy of Dermatology and Venereology (EADV). J Eur Acad Dermatol Venereol. 2020 Aug 24. doi:10.1111/jdv.16752

Murrell DF, Dick S, Ahmed AR, et al. Consensus statement on definitions of disease, end points, and therapeutic response for pemphigus. J Am Acad Dermatol. 2008;58(6):1043–1046. doi:10.1016/j.jaad.2008.01.012

Hébert V, Boulard C, Houivet E, et al. Large international validation of ABSIS and PDAI pemphigus severity scores. J Invest Dermatol. 2019;139(1):31–37. doi:10.1016/j.jid.2018.04.042

Basset N, Guillot B, Michel B, Meynadier J, Guilhou JJ. Dapsone as initial treatment in superficial pemphigus. Report of nine cases. Arch Dermatol. 1987;123(6):783–785. doi:10.1111/j.1365 2133.1990.tb01446.x

Almugairen N, Hospital V, Bedane C, et al. Assessment of the rate of long term complete remission off therapy in patients with pemphigus treated with different regimens including medium and high dose corticosteroids. J Am Acad Dermatol. 2013;69(4):583–588. doi:10.1016/j.jaad.2013.05.016

Werth VP, Fivenson D, Pandya AG, et al. Multicenter randomized, double blind, placebo controlled, clinical trial of dapsone as a glucocorticoid sparing agent in maintenance phase pemphigus vulgaris. Arch Dermatol. 2008;144(1):25–32. doi:10.1001/archderm.144.1.25

Ingen Housz Oro S, Valeyrie Allanore L, Cosnes A, et al. First line treatment of pemphigus vulgaris with a combination of rituximab and high potency topical corticosteroids. JAMA Dermatol. 2015;151(2):200–203. doi:10.1001/jamadermatol.2014.2421

Joly P, Maho Vaillant M, Prost Squarcioni C, et al. First line rituximab combined with short term prednisone versus prednisone alone for the treatment of pemphigus (Ritux 3): a prospective, multicentre, parallel group, open label randomized trial. Lancet. 2017;389(10083):2031–2040. doi:10.1016/S0140 6736(17)30070 3

Joly P, Mouquet H, Roujeau J C, et al. A single cycle of rituximab for the treatment of severe pemphigus. N Engl J Med. 2007;357(6):545–552. doi:10.1056/NEJMoa067752

Cianchini G, Lupi F, Masini C, Corona R, Puddu P, De Pita O. Therapy with rituximab for autoimmune pemphigus: results from a single center observational study on 42 cases with long term follow up. J Am Acad Dermatol. 2012;67(4):617–622. doi:10.1016/j.jaad.2012.04.028

Beissert S, Mimouni D, Kanwar AJ, Solomons N, Kalia V, Anhalt GJ. Treating pemphigus vulgaris with prednisone and mycophenolate mofetil: a multicenter, randomized, placebo controlled trial. J Invest Dermatol. 2010;130(8):2041–2048. doi:10.1038/jid.2010.91

Beissert S, Werfel T, Frieling U, et al. A comparison of oral methylprednisolone plus azathioprine or mycophenolate mofetil for the treatment of pemphigus. Arch Dermatol. 2006;142(11):1447–1454. doi:10.1001/archderm.142.11.1447

Hébert V, Vermeulin T, Tanguy L, et al. Comparison of real costs in the French healthcare system in newly diagnosed patients with pemphigus for first line treatment with rituximab vs. standard corticosteroid regimen: data from a national multicentre trial. Br J Dermatol. 2019;183(1):121–127. doi:10.1111/bjd.18563

Chen DM, Odueyungbo A, Csinady E, et al. Rituximab is an effective treatment in patients with pemphigus vulgaris and demonstrates a steroid sparing effect. Br J Dermatol. 2019;182(5):1111–1119. doi:10.1111/bjd.18482

Jelti L, Prost Squarcioni C, Ingen Housz Oro S, et al. Update of the French recommendations for the management of pemphigus. Ann Dermatol Venereol. 2019;146(4):279–286. doi:10.1016/j.annder.2019.01.018

Mignard C, Maho Vaillant M, Golinski ML, et al. Factors associated with short term relapse in patients with pemphigus who receive rituximab as first line therapy: a post hoc analysis of a randomized clinical trial. JAMA Dermatol. 2020;156(5):545–552. doi:10.1001/jamadermatol.2020.0290

Colliou N, Picard D, Caillot F, et al. Long term remissions of severe pemphigus after rituximab therapy are associated with prolonged failure of desmoglein B cell response. Sci Transl Med. 2013;5(175):175ra30. doi:10.1126/scitranslmed.3005561

Sanchez J, Ingen Housz Oro S, Chosidow O, Antonicelli F, Bernard P. Rituximab as single long term maintenance therapy in patients with difficult to treat pemphigus. JAMA Dermatol. 2018;154(3):363–365. doi:10.1001/jamadermatol.2017.5176

Werth VP. Treatment of pemphigus vulgaris with brief, high dose intravenous glucocorticoids. Arch Dermatol. 1996;132(12):1435–1439. doi:10.1001/archderm.132.12.1435

Amagai M, Ikeda S, Shimizu H, et al. A randomized double blind trial of intravenous immunoglobulin for pemphigus. J Am Acad Dermatol. 2009;60(4):595–603. doi:10.1016/j.jaad.2008.09.052

Behzad M, Möbs C, Kneisel A, et al. Combined treatment with immunoadsorption and rituximab leads to fast and prolonged clinical remission in difficult to treat pemphigus vulgaris. Br J Dermatol. 2012;166(4):844–852. doi:10.1111/j.1365 2133.2011.10732.x

Kasperkiewicz M, Shimanovich I, Meier M, et al. Treatment of severe pemphigus with a combination of immunoadsorption, rituximab, pulsed dexamethasone and azathioprine/mycophenolate mofetil: a pilot study of 23 patients. Br J Dermatol. 2012;166(1):154–160. doi:10.1111/j.1365 2133.2011.10585.x

Meggitt SJ, Anstey AV, Mohd Mustapa MF, Reynolds NJ, Wakelin S. British Association of Dermatologists’ guidelines for the safe and effective prescribing of azathioprine. Br J Dermatol. 2011;165(4):711–734. doi:10.1111/j.1365 2133.2011.10575.x

Abasq C, Mouquet H, Gilbert D, et al. ELISA testing of anti desmoglein 1 and 3 antibodies in the management of pemphigus. Arch Dermatol. 2009;145(5):529–535. doi:10.1001/archdermatol.2009.9

Downloads

Published

2025-06-12

Issue

Section

Guidelines