Life-threatening transient neonatal Behçet's disease

This case report describes transient neonatal Behçet's disease, with life-threatening complications in the neonate. Male Baby R developed blood-streaked diarrhoea 5 days after birth, followed by recurrent severe scarring orogenital ulceration and vasculitic skin lesions. In this sixth week of life, he developed stridor leading to a respiratory arrest and necessitating assisted ventilation. No infective cause was isolated. Baby R responded well to i.v. and subsequent oral steroid therapy. At 8 weeks old he had fully recovered and remains well. Baby R's mother was not previously known to have Behçet's disease. During the pregnancy, she began to suffer orogenital ulceration, associated with skin lesions typical of Behçet's disease. Mild orogenital ulceration has become recurrent.