Thalidomide responsiveness in an infant with Behçet's syndrome.

Abstract

Behçet's syndrome is a chronic relapsing vasculitis characterized by aphthous stomatitis, genital ulcerations, and uveitis. The disease is a multisystem illness, which may involve the skin, joints, gastrointestinal tract, blood vessels, central nervous system, and other organs. We report a case of an infant with features of Behçet's syndrome who did not respond to conventional immunosuppression with high-dose corticosteroids and cytotoxic agents. Thalidomide, a potent immune response-modifying drug, was used with marked improvement and resolution of symptoms. This finding supports a trial of thalidomide treatment in patients with Behçet's syndrome who are unresponsive to other forms of immunosuppressive therapy or develop undesirable side effects.