title: Interleukin 4 inhibition as a potential therapeutic in pemphigus.
abstract: Pemphigus is an autoimmune bullous skin disease that results from desmosomal protein desmoglein 3 and 1 loss in pemphigus vulgaris and foliaceus, respectively. It can be considered as a Th2-dominant disease over-expressed by Th2 cell cytokines. Interleukin (IL)-4 is a key cytokine which can exacerbate Th2 over-expression in addition to isotype switching to immunoglobin (Ig)G1 and IgG4 that are responsible for desmoglein loss. Elevation of IL-4 level has also been reported in various studies. Considering the important role of IL-4 in severe phase of pemphigus and lack of effective and safeness therapy for this potentially fatal disease, anti-IL-4 therapy was introduced as a potential curative for pemphigus disease. This study reviewed all studies about any roles of IL-4 that can directly and indirectly be played in the development of pemphigus and IL-4 inhibition with interferons and dupilumab therapy were introduced as a novel pemphigus treatment for patients who are in relapse phase of the disease. Dupilumab was also introduced as a possible treatment for patients with severe pemphigus. It can directly inhibit IL-4 by targeting IL-4 α-chain receptor. IL-4 inhibition can lead to the creation of Th1:Th2 balance by various pathways, discussed in this study.
authors.affiliation: Bullous Diseases Research Center, Department of Dermatology, Razi Hospital, Tehran, Iran. Electronic address: soheil.tavakolpour@gmail.com.
authors.forename: Soheil
authors.initials: S
authors.lastname: Tavakolpour
authors.affiliation: Stem Cell Technology Research Center, Tehran, Iran.
authors.forename: Vahid
authors.initials: V
authors.lastname: Tavakolpour
mesh_terms: D000911:Antibodies, Monoclonal / Q000276:immunology / Q000627:therapeutic use; D061067:Antibodies, Monoclonal, Humanized; D002454:Cell Differentiation / Q000187:drug effects / Q000276:immunology*; D006801:Humans; D017578:Immunoglobulin Class Switching / Q000276:immunology; D007074:Immunoglobulin G / Q000276:immunology / Q000378:metabolism; D015847:Interleukin-4 / Q000037:antagonists & inhibitors / Q000276:immunology* / Q000378:metabolism; D018448:Models, Immunological; D010392:Pemphigus / Q000188:drug therapy / Q000276:immunology* / Q000378:metabolism; D019948:Receptors, Interleukin-4 / Q000037:antagonists & inhibitors / Q000276:immunology; D018418:Th2 Cells / Q000187:drug effects / Q000276:immunology* / Q000378:metabolism
chemical_list: D000911:Antibodies, Monoclonal; D061067:Antibodies, Monoclonal, Humanized; C508598:IL4 protein, human; D007074:Immunoglobulin G; D019948:Receptors, Interleukin-4; D015847:Interleukin-4; C582203:dupilumab
keywords: Anti-cytokine therapy; Autoimmune disease; Interleukin-4; Pemphigus