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Review
. 2025 Feb;39(2):290-300.
doi: 10.1111/jdv.20313. Epub 2024 Sep 19.

Bullous pemphigoid burden of disease, management and unmet therapeutic needs

V P Werth  1   2 , D F Murrell  3 , P Joly  4 , M Ardeleanu  5 , V Hultsch  6
Affiliations
Review

Bullous pemphigoid burden of disease, management and unmet therapeutic needs

V P Werth et al. J Eur Acad Dermatol Venereol. 2025 Feb.

Abstract

Bullous pemphigoid (BP) is an autoimmune blistering disease that can have a profound negative impact on quality of life. BP most often affects the elderly, a population with a high medical burden and special safety concerns. In this review, we outline the BP disease course, diagnosis, epidemiology and comorbidities, and describe tools commonly used to assess BP disease activity and severity and the impact of BP on health-related quality of life. We also outline biologic treatments currently under investigation for the treatment of BP and highlight the importance of considering safety when treating elderly patients.

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Conflict of interest statement

VPW has received honoraria and research grant funding from Regeneron Pharmaceuticals Inc. and is a co‐creator of BPDAI. DFM has been an investigator and/or advisory board member for Argenx, Principia Biopharma, Regeneron Pharmaceuticals Inc. and Roche, has been an investigator for AstraZeneca and is a co‐creator of BPDAI. PJ has been a consultant for Amgen, Argenx, AstraZeneca, Innovaderm, Principia Biopharma, Roche, Sanofi‐Regeneron Pharmaceuticals Inc. and Thermo Fisher Scientific. MA is an employee and shareholder of Regeneron Pharmaceuticals Inc. VH is an employee of Sanofi and may hold stock and/or stock options in the company.

Figures

FIGURE 1
FIGURE 1
Photos of patients with BP showing different clinical features and severity of the disease. (a) Multiple blood‐filled bullae on the foot; (b) urticarial plaques, some studded with tense bullae or erosions on the flexor surface of the left thigh and external knee; (c) large bullous lesion on leg; (d) erythematous urticarial plaques on the trunk; (e) erythematous urticarial macules on the anterior trunk; (f) haemorrhagic vesicles on the palms.
FIGURE 2
FIGURE 2
Treatment ladder for BP*. Syntax for recommendations: Recommendations from large randomized prospective multicentre studies ‘is recommended’; recommendations from small randomized or non‐randomized prospective multicentre or large retrospective multicentre studies ‘may be recommended’; recommendations pending from case series, or small retrospective single‐centre studies ‘may be considered’; ‘may be considered’ is used when a consensus could not be reached among experts. BP, bullous pemphigoid; BPDAI, bullous pemphigoid disease area index; IVIG, intravenous immunoglobulin; OCS, oral corticosteroids; TCS, topical corticosteroids. *Based on the updated S2K guidelines for the management of bullous pemphigoid. Figure adapted from Borradori et al.
FIGURE 3
FIGURE 3
Summary of BP therapeutic treatments. CCL11, C‐C motif chemokine ligand 11; FcRn, neonatal Fc receptor; IgG, immunoglobulin G; IgE, immunoglobulin E; IL, interleukin; JAK, Janus kinase; Th2, T helper 2; Th17, T helper 17. Figure adapted from Haddad et al.
See this image and copyright information in PMC

References

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