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A Review Comparing International Guidelines for the Management of Bullous Pemphigoid, Pemphigoid Gestationis, Mucous Membrane Pemphigoid, and Epidermolysis Bullosa Acquisita

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Abstract

Autoimmune blistering disease management can be challenging as treatment modalities vary greatly and no single standard of care exists. We consolidated the recommendations of international management guidelines in order to provide optimal management suggestions to physicians. A comprehensive literature search in PubMed/MEDLINE for published blistering disease management guidelines and consensus statements was conducted in November 2019. Search terms included “guideline or guidelines” or “consensus” and “pemphigoid” or “autoimmune blistering disease” or “epidermolysis bullosa acquisita”. We included guidelines from established dermatologic societies and expert consensus groups. We excluded literature reviews, guidelines established by an association without dermatologists, or those specific to a single treatment. Guidelines in all languages were considered. Eleven guidelines from dermatologic associations and consensus groups meeting our inclusion criteria were selected. Several differences between recommendations, most notably when to introduce adjuvants for refractory disease, were found in bullous pemphigoid. In mucous membrane pemphigoid, treatment was directed to the sites involved and managed with systemic corticosteroids and immunosuppressants/biologics. There was no universal consensus on the first-line treatment for epidermolysis bullosa acquisita, but a combination of immunosuppressive, anti-inflammatory, and anti-neutrophil therapy was utilized. Comparison of the management guidelines revealed underrepresentation of guidelines from developing nations and key differences between the management styles among dermatologists from Europe and Asia. We attribute these discrepancies to the time elapsed between guidelines, regional differences, and demands of the local healthcare systems.

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Author notes

  1. Payal M. Patel and Virginia A. Jones contributed equally as first author.

Authors and Affiliations

  1. Department of Dermatology, University of Illinois at Chicago, 808 S. Wood St, RM377, Chicago, IL, 60612, USA

    Payal M. Patel, Virginia A. Jones, Taryn N. Murray & Kyle T. Amber

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  1. Payal M. Patel
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Correspondence to Kyle T. Amber.

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This study has not been funded by any sources.

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Payal M. Patel, Virginia A. Jones, Taryn N. Murray, Kyle T. Amber declare that they have no conflicts of interest.

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Patel, P.M., Jones, V.A., Murray, T.N. et al. A Review Comparing International Guidelines for the Management of Bullous Pemphigoid, Pemphigoid Gestationis, Mucous Membrane Pemphigoid, and Epidermolysis Bullosa Acquisita. Am J Clin Dermatol 21, 557–565 (2020). https://doi.org/10.1007/s40257-020-00513-3

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