Lacrimo-auricolo-dento-digital syndrome mimicking primary juvenile Sjögren's syndrome

We describe a 24 year old female patient affected with symptoms of severe xerostomia and keratoconjunctivitis sicca since her childhood. She also had several malformations involving face and digits as well as cup shaped ears, enamel dysplasia and absence of lacrimal puncta. Hence, the diagnosis of Lacrimo-Auricolo-Dento-Digital (LADD) syndrome was made. The main differences between this rare, inherited disease and primary juvenile Sjögren's syndrome are pointed out.