Behçet syndrome in children

Abstract

Behçet syndrome (BS) may be occasionally seen in children; its frequency ranges between 4 and 26% among the whole BS population. Genders are equally affected, and familial cases are more frequent than in adult patients. The mean age of onset is around 8 years, but the disease is generally recognized later (3-5 years). While erythema nodosum is more prevalent among those with prepubertal onset, genital ulcers develop more frequently after puberty. Skin mucosa lesions and vascular disease are less frequent, while neurological disease is more common among pediatric patients compared to the adults. Despite its low frequency, large vessel disease is still severe and is the main cause of mortality. Dural sinus thrombosis is the dominating type of neurological disease. Genetic conditions mimicking BS may be misleading and investigated particularly in this age group, especially in patients with familial history, recurrent fevers with elevated acute phase reactants, and prominent colitis. Similar to what is observed in adults, disease runs significantly more severe course among boys with regard to morbidity and mortality.