An Incomplete Form of Childhood Behçet's Disease Treated with Infliximab

Abstract

Behçet's disease (BD) is a multi-systemic vasculitis characterized by the possible presence of cutaneous, ocular, articular and neurological manifestations. In this report, we examine the case of a fifteen-year old boy with an incomplete form of juvenile Behçet's disease which began with joint involvement and developed into a complete form only after several years. The patient showed a rapid response to anti-TNF-alpha (infliximab) with an improvement of mucocutaneous lesions (oral and genital ulcers, pseudofolliculitis) and arthritis.

Behçet's disease with multiple splenic abscesses in a child

“…In 2007, Kobayashi et al16 suggested treatment standards based on disease severity, including 5-aminosalicylic acid, steroids, immunosuppressive drugs, enteral nutrition, total parenteral nutrition, and surgical resection as well as experimental treatments such as infliximab, colchicine, leukocytapheresis, thalidomide, antibiotics, and endoscopic ethanol spray. Similar treatments were reported for childhood intestinal BD, and symptoms reportedly resolved with infliximab for steroid-resistant (refractory) BD 1718…”

Calf Pain as a Prominent Feature of Behçet's Disease

“…In some instances, patients are managed as incomplete BD, where treatment is often initiated before the clinical criteria are fulfilled less the disease progressed irreversibly. [8][9][10] In these four cases, a diagnosis of incomplete BD was made based on the following: the presence of skin lesions and oral ulcers, the suspicion of a vasculitic process underlying the leg pain and skin manifestations, and the absence of other systemic findings that would fulfill the classification criteria of another connective tissue disease or systemic vasculitis. In this case series, however, oral ulcers were not even among the main complaint and were discovered only on probing.…”