Neonatal Behçet's disease

A male infant of 36 weeks gestation was delivered by emergency caesarean section due to suboptimal control of maternal Behçet's disease (BD). His mother had a 15- year history of BD with oral and genital ulcers, erythema nodosum and cutaneous vasculitis. She took oral prednisolone and azathioprine during pregnancy. At birth, the baby was well and admitted to transitional care to establish formula feeding. On day 8, he developed napkin dermatitis. Despite topical treatment, this worsened and pustulonecrotic lesions developed in the perianal region (figure 1). Herpetic infection was suspected. The baby was commenced on intravenous antibiotics and aciclovir. Blood tests were unremarkable and viral and bacterial cultures were negative. On day 10, mouth ulcers were noted together with pathergy at cannulation sites (figures 2 and 3). There was no joint swelling or eye involvement. A diagnosis of neonatal BD was made. He was given paracetamol for pain but required no further treatment and the lesions gradually resolved. Corticosteroids would have been given if oral ulceration interfered with feeding, if anal ulceration led to infection or if there was any evidence of serious organ involvement.