ABSTRACT
Introduction: Epidermolysis bullosa acquisita (EBA) is a chronic autoimmune bullous dermatosis (AIBD). Treatment of EBA is challenging and mostly relies on systemic immunosuppression. During the last decade, intensive research led to the identification of new potential therapeutic targets that interfere in different phases of disease progression. Therapeutic interventions acting upon these candidate drug targets in animal models of EBA, such as cytokine-modulating biologics and small molecules, have validated them as potential new therapeutic strategies for EBA patients.
Areas covered: In this paper, we review the current treatments for EBA, describe the pathogenesis of the disease, and finally specify new drug candidates for the development of a more specific therapy with minimized side-effects for EBA and potentially other autoimmune diseases.
Expert opinion: We currently understand EBA as a disease that evolves from the interplay of many different signaling pathways. These signaling pathways, which are described in this review, provide new targets for EBA treatment. The ultimate goal of this research field is the development of specific, pathogenesis-based therapeutic strategies. Through identification of up- or downregulated pathways that dominate disease progression in individual patients, we expect therapy in EBA to become more and more precise and move towards a patient-based therapy.
Article highlights
Epidermolysis bullosa acquisita is a difficult-to-treat autoimmune bullous disease with autoantibodies targeting COL7, the major component of anchoring fibrils
Treatment of EBA is performed using standard immunosuppressive therapy that is accompanied by adverse effects
Pathogenesis of EBA is divided into (i) the generation of autoantibodies, (ii) the maintenance of autoantibodies in the circulation, and (iii) autoantibody-induced tissue injury
Based on the latest knowledge of signaling pathways and mechanisms that lead to EBA, we here provide novel therapeutic targets that might enhance the development of more specific, personalized medicine.
New-discovered therapeutic options of EBA can be transferred to in other antibody-driven autoimmune diseases such as RA, EAE and multiple sclerosis, since signaling pathways and inflammation in EBA analogously happen in those diseases
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Financial and competing interests disclosure
The authors have no relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript. This includes employment, consultancies, honoraria, stock ownership or options, expert testimony, grants or patents received or pending, or royalties.