Setleis syndrome - Bitemporal "forceps marks" syndrome in a 6-day-old Taiwanese infant

Setleis syndrome, a variant of aplasia cutis congenita, is a rare genetic disease characterized by bitemporal scarring resembling forceps marks, abnormal eyebrows and eyelashes, low frontal hairline and 'pursed lips'. We report the first Taiwanese case in a 6-day-old full-term male infant who was born spontaneously to unrelated, normal-appearing parents with atrophic scar-like lesions on the bilateral temporal areas. In addition, upward slanting of the eyebrows and partial absence of the lateral brows were found. There were no other obvious development anomalies. Low frontal hair line and 'pursed lips' appearance became more apparent at 6-month of age. The baby was otherwise normal, alert and active. The clinical features are consistent with Setleis syndrome.