The effectiveness of adalimumab as an add-on therapy in two cases with leg ulcers in Behçet's disease resistant to conventional immunosuppressive therapy alone and a review of the literature.

Abstract

The current report presents two cases with leg ulcers related to Behçet's disease (BD) resistant to conventional immunosuppressive therapy (CIST) but successfully treated with adalimumab (ADA). BD, which can affect vessels of any size and type, is a systemic vasculitis. In the vascular system, veins are the most predominantly affected blood vessels, with deep vein thrombosis and recurrent superficial vein thrombophlebitis being the most common vascular signs of the disease in the lower extremities. Leg ulcers, commonly associated with vasculitis or deep vein thrombosis, are rare in patients with BD. Conventional immunosuppressive therapy is very critical to prevent relapses and diminish the risk of post-thrombotic syndrome. In patients with BD-associated venous thrombosis (deep vein thrombosis or superficial vein thrombophlebitis) resistant to these treatments, tumour necrosis factor-α inhibitors can be used alone or in combination with traditional disease-modifying antirheumatic drugs. In view of such information, add-on adalimumab treatment was considered appropriate for both patients. Response to this intervention was highly satisfying for the patients at the end of the 6-month treatment. Nonetheless, it warrants further studies directly evaluating the efficacy of tumour necrosis factor-α inhibitors alone in leg ulcers in BD.