AUTHOR=Toh Wu Han , Lee Hua-En , Chen Chun-Bing 

TITLE=Targeting type 2 inflammation in bullous pemphigoid: current and emerging therapeutic approaches

JOURNAL=Frontiers in Medicine

VOLUME=Volume 10 - 2023

YEAR=2023

URL=https://www.frontiersin.org/journals/medicine/articles/10.3389/fmed.2023.1196946

DOI=10.3389/fmed.2023.1196946

ISSN=2296-858X

ABSTRACT=Bullous pemphigoid (BP) is one of the most common autoimmune bullous diseases, and mainly affects an elderly popula�on with mul�morbidity. Exis�ng treatment op�ons for BP are mainly cor�costeroids and immunosuppressants, which can have substan�al side effects on these vulnerable pa�ents that even exceed their therapeu�c benefits. The blisters associated with BP result from IgG and IgE autoan�bodies binding to the central components of hemidesmosome, BP180 and BP230, s�mula�ng a destruc�ve inflammatory process. The known characteris�c features of BP, such as intense pruritus, ur�carial prodrome, peripheral eosinophilia, elevated IgE, as well as recent expanding evidence from in vitro and in vivo studies implicate Type 2 inflamma�on as an important driver of BP pathogenesis. Type 2 inflamma�on is an inflammatory pathway involving a subset of CD4+ T cells that secrete IL-4, IL-5, and IL-13, IgE-secre�ng B cells, and granulocytes, such as eosinophils, mast cells and basophils. It is believed that effectors in Type 2 inflamma�on may serve as novel and effec�ve treatment targets for BP. This review focuses on recent understandings of BP pathogenesis with a par�cular emphasis on the role of Type 2 inflamma�on. We summarize current clinical evidence of using Rituximab (B cell deple�on), Omalizumab (an�-IgE an�body), and Dupilumab (an�-IL-4/13 an�body) in the treatment of BP. Latest advances in emerging targeted therapeu�c approaches for BP treatment are also discussed.