Behçet's syndrome treated with infliximab, which caused a palmoplantar pustulosis, subsequently maintained on low-dose etanercept

B ehçet’s syndrome is a chronic, relapsing, multisystemic, inflammatory disorder. The diagnosis of Behçet’s syndrome is based on clinical criteria and no pathognomonic laboratory findings exist. A diagnosis is made by the presence of recurrent oral ulceration, the hallmark of this disease, plus any two of the following: recurrent genital ulcerations, ocular lesions (anterior or posterior uveitis, cells in vitreous or slit lamp examination, or retinal vasculitis), typical skin lesions, and a positive pathergy (skin hyperreactivity) test (1). Inflammation in Behçet’s disease is thought to be mediated by cytokines derived from T-helper type 1 lymphocytes, including tumor necrosis factor (TNF) (2). In the absence of the multisystem disease, aphthae may be treated topically with topical steroid, topical tacrolimus, and/or with topical lidocaine (3). Mucocutaneous manifestations may respond to colchicine or dapsone alone or in combination (3). Oral corticosteroid may not control severe disease where upon methotrexate (4), azathioprine (5) cyclosporine (6), tacrolimus (7), chlorambucil (4), or cyclophosphamide (4) need to be considered. Thalidomide can be used with significant clinical benefit but is frequently complicated by peripheral neuropathy (8). Interferon a is also an effective alternative treatment for the mucocutaneous manifestations (9). Recently, the anti-TNF-a agents have successfully been used in treating resistant Behçet’s syndrome. Etanercept, a human TNF receptor fusion protein, has shown to be beneficial for the resistant cases at a dose of 25 mg twice weekly (2). Infliximab, a chimeric monoclonal antibody against TNF-a, has also been used effectively in therapy-resistant Behçet’s disease, including cases resistant to etanercept (10).