Prosthodontic Management of Children with Ectodermal Dysplasia: Review of Literature

Introduction:

Ectodermal dysplasia (ED) is a large group of heterogeneous heritable conditions characterized by congenital defects of two or more ectodermal structures and their appendages: hair (hypotrichosis, partial or total alopecia), nails (dystrophic, hypertrophic, abnormally keratinized), teeth (enamel defect or absent) and sweat glands (hypoplastic or aplastic). Thurnam published the first report of a patient with ectodermal dysplasia in 1848. The term ectodermal dysplasia was not coineduntil 1929. In 1971 only eight forms of ED were known. Now approximately 200 different ED have been delineated; about 30 have been identified at the molecular level with identification of the causative.gene. 

Classification:

There are several classifications given by different authors. Some are based on clinical features and others on genetic component of the disorder. Clinically there are two major types of EDnamely hidrotic and anhidrotic (hypohidrotic form). The hidrotic form, inherited as an autosomal trait, affects teeth, hair and nails but usually spares the sweat glands and was first described by Clouston in 1929. Whereas the hypohidrotic form (Christ-Siemens-Touraine Syndrome) is most common type seems to be an X-linked recessive trait, with an incidence of this syndrome estimated to be 1 to 7 per 10,000 live births.Hypodontia, hypotrichosis and hypohidrosis which form a triad are the characteristic feature of the hypohidrotic form.