Systemic Treatments for Mucocutaneous Behçet’s Disease

Abstract

The treatment of Behçet’s disease (BD) is primarily symptomatic and aims at suppressing acute inflammation, preventing relapses, damage, and complications. The therapeutic approach is customized for each patient based on observed manifestations and their severity. While mucocutaneous lesions, the most prevalent manifestations of the disease, are not life threatening, they significantly impact the quality of life, particularly in patients experiencing severe and frequent recurrences. This chapter provides an overview of current approaches to treating mucocutaneous lesions of BD, encompassing both traditional and emerging therapeutic strategies. Colchicine is considered as the first-line therapy for mucocutaneous BD. Systemic steroids are typically employed for short-term acute inflammation suppression. In cases resistant to colchicine, conventional treatments such as azathioprine, dapsone, interferon-α, and thalidomide may be considered. Apremilast has demonstrated effectiveness in treating oral aphthous ulceration in randomized controlled trials. Tumor necrosis factor-alpha inhibitors, commonly used for major organ involvement resistant to conventional immunosuppressive therapies, should be reserved for severe mucocutaneous cases refractory to standard therapies. Recent advances, particularly the introduction of targeted biologic agents, present novel opportunities for more precise and effective interventions in BD. © The Editor(s) (if applicable) and The Author(s), under exclusive license to Springer Nature Switzerland AG 2025.