Abstract
Autoimmune bullous dermatoses (AIBDs) are a group of rare chronic inflammatory skin diseases, which clinically manifest as blisters and erosions of the skin and/or mucosa. Immunologically, AIBDs are characterized and caused by autoantibodies targeting adhesion molecules in the skin and mucosa. According to the histological location of the blistering, AIBDs are classified into the following two main subtypes: pemphigus (intraepidermal blistering) and pemphigoid (subepidermal blistering). Most AIBDs were potentially life-threatening diseases before the advent of immunosuppressive drugs, especially systemic steroid therapies, which suppress pathogenic immunological activity. Although there have been recent advancements in the understanding of the pathogenesis of AIBDs, glucocorticosteroids and/or adjuvant immunosuppressive drugs are still needed to control disease activity. However, the long-term use of systemic immunosuppression is associated with major adverse events, including death. Based on the growing understanding of AIBD pathogenesis, novel treatment targets have emerged, some of which are currently being evaluated in clinical trials. Within this article, we review the current clinical trials involving pemphigus and pemphigoid and discuss the rationale that lead to these trials. Overall, we aim to foster insights into translational research in AIBDs to improve patient care.
Highlights
Autoimmune bullous dermatoses (AIBDs) are a heterogeneous group of skin diseases that are characterized and caused by autoantibodies targeting adhesion molecules in the skin and/or mucous membranes
To foster translational AIBD research, in this article, we summarize the current clinical trials involving pemphigus and pemphigoid diseases
Platelet-rich plasma (PRP) resulted in clinical improvement, as in the previous study, there were no significant differences between PRP and intralesional steroid injections [62]
Summary
Autoimmune bullous dermatoses (AIBDs) are a heterogeneous group of skin diseases that are characterized and caused by autoantibodies targeting adhesion molecules in the skin and/or mucous membranes. The linear deposition of autoantibodies along the dermal-epidermal junction causes subepidermal blistering, resulting in tense blisters. Based on the target molecules of the autoantibodies and the clinical manifestations, pemphigoid diseases are classified as bullous pemphigoid (BP), mucous membrane pemphigoid (MMP), epidermolysis bullosa acquisita (EBA), anti-laminin-γ1/p200 pemphigoid (p200), pemphigoid gestationis (PG), lichen planus pemphigoides (LPP), linear IgA bullous dermatosis (LAD), and dermatitis herpetiformis (DH), which is associated with gluten-sensitive enteropathy and characteristic granular IgA deposits in the upper dermis [3,4,5]. The advent of systemic steroid therapy significantly improved the prognosis of AIBDs, these groups of diseases are still potentially life-threatening, mainly due to adverse events resulting from corticosteroid treatment [6]. The development of alternative treatment modalities that have fewer adverse events is urgently needed for the treatment of AIBD patients
Sign-in/Register to view highlights & summary Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a callDisclaimer: All third-party content on this website/platform is and will remain the property of their respective owners and is provided on "as is" basis without any warranties, express or implied. Use of third-party content does not indicate any affiliation, sponsorship with or endorsement by them. Any references to third-party content is to identify the corresponding services and shall be considered fair use under The CopyrightLaw.
