Life-threatening transient neonatal Beh?et's disease

This case report describes transient neonatal Beh?et'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 Beh?et's disease. During the pregnancy, she began to suffer orogenital ulceration, associated with skin lesions typical of Beh?et's disease. Mild orogenital ulceration has become recurrent.