Behcet's disease in UK children: clinical features and treatment includingthalidomide

Objective, To study the clinical spectrum of Behcet's disease (BD) in child hood, and to report our experience of using thalidomide. Method. Ten children, diagnosed with BD, were studied retrospectively. Results. The median (range) age at first presentation was 4 (1.2-12.0) yr, at diagnosis was 11 (3-15) yr and the follow-up period was 4.1 (0.6-6.3) yr . Oral ulcers were present in all patients (100%), genital ulcers were pres ent in six (60%), peri-anal ulcers were present in three (30%), skin manife stations were present in nine (90%), intracranial hypertension was present in two (20%), mild gastrointestinal symptoms were present in five (50%), jo int symptoms were present in six (60%), ocular lesions were present in five (50%), but only one child had anterior and posterior uveitis. Therapeutica lly, a range of drugs was used, including colchicine, that resulted in good responses in five children. Thalidomide (1 mg/kg/week to 1 mg/kg/day) was used in five children who were unresponsive to other immunosuppressive agen ts. It resulted in complete remission in three children and less frequent m ilder oral ulcers in two. Neuropathy developed in two children and in one i t was irreversible. Conclusion. BD in children is similar to the disease in adults. Thalidomide provided a useful therapeutic option for severe oral and genital ulceratio n which was unresponsive to other therapies. Awareness of the danger of axo nal neuropathy and teratogenesis at all times during thalidomide therapy is crucial. A low dose is probably as effective as higher doses.