Systemic Inherited Disorders with Dental Involvement

This chapter discusses systemic inherited disorders with dental involvement. It discusses acroosteolysis, amelocerebrohypohidrosis syndrome, cryptodontic brachymetacarpalia, hypodontia and nail dysgenesis, hypophosphatasia, hypophosphatemic vitamin D-resistant rickets, hypoplastic-hypocalcified enamel, onycholysis, functionali hypohidrosis, incontinentia pigmenti, oculodentoosseous dysplasia, otodental syndrome, pseudohypoparathyroidism and pseudo-pseudohypoparathyroidism, and rothmund−thomson syndrome among others. Acroosteolysis, which has an autosomal dominant mode of inheritance, is characterized by short stature, disintegration of the terminal phalanges of fingers and toes, abnormal skull shape, and premature loss of teeth. The Amelocerebrohypohidrosis syndrome is characterized by the following features: (1) severe seizures, (2) progressive mental retardation, (3) muscular spasticity, (4) hypohidrosis, and (5) enamel hypoplasia. There is marked hypoplasia of enamel, resulting in complete absence, except for small zones of thin enamel at cervix of the tooth crowns. The enamel defect is generalized and involves all teeth in both primary and secondary dentitions.