Behçet's disease in children, an overview.

BD is a systemic inflammatory disease with a variable vasculitis. Paediatric onset is very rare and carries a strong genetic component. Oral ulcers and fever of unknown origin are frequent at onset and difficult to distinguish from other inflammatory disorders; therefore, expert opinion is still mandatory to recognize the disease early. An international expert consensus has recently proposed new classification criteria for children with BD. The clinical spectrum of BD is heterogeneous and influenced by gender, ethnicity and country of residence. Young males have the worst prognosis with significantly more frequent neurological, ocular and vascular involvement. BD treatment is aimed at alleviating inflammation. Among all drugs, TNFα inhibitors have become a standard to control severe ocular, neurological and digestive system involvement.