[Enamel defects in tuberous sclerosis: hard facts of diagnostic importance]

Tuberous sclerosis is a complex syndrome. Recognition of the disease can be very difficult because of its incomplete forms. In this article a new symptom of possibly great diagnostic value is discussed, namely the presence of enamel defects in patients with tuberous sclerosis. For a clear understanding and correct interpretation of enamel defects in general, the role of the odontogenesis, the amelogenesis and the different types of developmental disturbances is explained. A review is given of all published investigations concerning the presence of enamel defects in tuberous sclerosis. The conclusion is that the enamel defects may be of great importance for an early diagnosis as well as for a possible revise of the penetrance and mutation rate of the disease.