Mucocutaneous Involvement in Behçet’s Disease: How Systemic Treatment Has Changed in the Last Decades and Future Perspectives

Abstract

Behçet's disease (BD) is a multisystemic disorder of unknown etiology characterized by the “triple symptom complex” consisting of recurrent oral aphthosis, genital ulcers, and chronic relapsing bilateral uveitis. Recurrent mucocutaneous lesions are generally considered the hallmark of the disease, being the most common symptoms presenting at the onset of disease. Although the improvement of knowledge about the pathogenetic mechanism added important changes in the treatment management of BD clinical manifestations, thus avoiding the appearance of serious life-threatening complications which are disease related, the mucocutaneous lesions are still the most nagging clinical manifestations to be treated. In this work we reviewed the current state of knowledge regarding the therapeutic approaches for mucocutaneous lesions of BD mainly based on controlled studies to provide a rational framework for selecting the appropriate therapy for treating these troublesome features of the disease.

Mucocutaneous Findings in Behçet’s Disease

“…Oral and genital ulcers can be treated with topical and systemic treatments [3,[147][148][149][150][151][152]. In recurrent OU with or without GU, systemic colchicine (1-2 mg/day) must be started as a first choice of treatment [147][148][149].…”

“…Thalidomide is another choice of therapy in recalcitrant OU and/or GU. However, it is not preferred due its high toxic effects [147,149,150]. Pentoxifylline, dapson, zinc sulphate, IFN-α and rebamipide are other alternative treatments worth for trying in OU.…”

“…Especially in female cases with GU and EN-like lesions, the combination of colchicine and benzathine penicillin is recommended [148]. This treatment combination has also been reported to decrease the frequency and duration of both OU and EN-like lesions [150]. IFN-α has been reported to decrease not only the frequency of GU and EN-like lesions but also the number of PPL [151].…”

“…Anti-TNF drugs are being used with success in patients with refractory mucocutaneous manifestations [151,152]. Recent studies of interleukin-1 (IL-1) inhibitors (anakinra, canakinumab) have demonstrated efficacy in OU and GU resistant to conventional therapy [150]. Finally, apremilast, phosphodiesterase 4 inhibitor, has been reported to be effective in treating OU and GU [150,152].…”

“…Recent studies of interleukin-1 (IL-1) inhibitors (anakinra, canakinumab) have demonstrated efficacy in OU and GU resistant to conventional therapy [150]. Finally, apremilast, phosphodiesterase 4 inhibitor, has been reported to be effective in treating OU and GU [150,152]. The treatment options can be seen more detailed in Table 1.…”