Fig. 1.

Dental phenotypes associated with an autosomal form of hypohidrotic ectodermal dysplasia (HED), linked to EDAR mutation. Clinical phenotype associated with a 10-year-old boy includes oligodontia (agenesis of mandibular permanent incisors and maxillary lateral incisors) and conical-shaped canines (a). A panoramic radiograph (b) of another patient (7 years old) with autosomal HED shows mandibular anodontia and maxillary oligodontia. There is agenesis of the primary right central incisor, lateral incisors, left canine and first primary molars with presence of only one dysmorphic permanent right central incisor germ and permanent first molars (b). Clinical (c) and radiographic (d) appearance of another patient (5 and 1/2 years old) with an autosomal dominant form of HED. Agenesis of maxillary and mandibular primary incisors, first molars and second maxillary right primary molar is evident. There is also agenesis of all permanent tooth germs, except for the permanent maxillary and mandibular first molars.