A proposed classification for heritable human dentine defects with a description of a new entity

A newly recognized heritable dentine defect is described which is manifested as an amber, translucent colouration and total pulpal obliteration in all primary teeth. Permanent teeth have a thistle-tube pulp configuration with ubiquitous pulp stones and normal colouration.The unusual finding of true denticles in these heritable dentine defects may represent the induction of odontoblastic differentiation by aberrant epigenetic factors produced in the abnormally developing dentine.A classification for the heritable dentine defects is proposed which consists of two distinguishable groups; the dentine dysplasias and the dentinogenesis imperfectas.