Navigating Bullous Pemphigoid: Consensus Recommendations for Diagnosis and Management-A Canadian Perspective.

Abstract

Bullous pemphigoid (BP) is an autoimmune disease of the skin characterized by subepidermal blistering accompanied by severe itch, causing a profound decrease in quality of life and conferring significant mortality risk. Because age is a primary risk factor, this rare condition is becoming more common as our population ages. Consequently, there is a need for enhanced recognition and appropriate management of BP. The objective of this endeavour was to develop a series of practical recommendations to improve the diagnosis and management of BP based on available evidence and expert opinion where evidence was lacking, with a focus on disease management within the Canadian context. A panel of 9 Canadian dermatologists with interest and experience in BP identified key topics in its diagnosis and management. A broad literature review, along with clinical expertise and opinion, supported the development of manuscript sections on each identified topic. Where appropriate, the panel developed clinically relevant recommendations that were adopted by consensus following a modified Delphi process and prespecified agreement cut-off of 80%. Panel members reviewed draft consensus statements and rated their level of agreement with each using an anonymous online survey platform. Statements not achieving consensus were discussed and updated in a live virtual meeting, after which another round of anonymous voting was held. Through this process, 18 recommendations were approved by the panel. These statements can guide healthcare providers in the practical management of BP in Canada and beyond.