Juvenile-onset Behçet's disease

Behçet's disease (BD) is a multi-system inflammatory disease of unknown aetiology characterised by recurrent painful mouth and genital ulcerations. It is a disease of young adults, with a peak age of onset of 25–30 years. Slightly more than one quarter of cases start before the age of 16 years when it is classified as juvenile-onset BD. The eyes are commonly affected and vision may be lost in up to half the patients. Vasculitis may affect vessels of any size but mainly veins, hence the term variable vessel vasculitis. Typical skin lesions include erythema nodosum, pseudofolliculitis or papulopustular lesions and acneiform nodules in post-adolescent patients not on corticosteroids. The bowel, nervous system and joints are also frequently affected. This short review highlights the important clinical features and provides simple advice for diagnosis and principles for management in younger individuals.