Hypohidrotic ectodermal dysplasia: A unique approach to esthetic and prosthetic management: A case report

Abstract

Hypohidrotic ectodermal dysplasia is a rare congenital disease that affects several ectodermal structures. The condition is usually transmitted as an x-linked recessive trait, in which gene is carried by the females and manifested in males. Manifestations of the disease differ in severity and involve teeth, skin, hair, nails and sweat and sebaceous gland. Ectodermal dysplasia is usually a difficult condition to manage. Prosthodontically, because of the typical oral deficiencies, and afflicted individuals are quite young to receive extensive prosthodontic treatment, which restores their appearance and helps them, for the development of positive self-image. This case report describes the management of upper jaw with over denture with copings on existing teeth i.e. two permanent peg shaped centrals as well as lateral incisors. However with adequate of retainer lower denture was provided with a new treatment modality.

Hereditary ectodermal dysplasia: A retrospective study

“…Thus, hypohydrotic ED is also known as Christ-Siemens-Touraine syndrome. [10] Clouston in 1939 used the term anhydrotic ED. [11] Felsher in 1944 changed the adjective anhydrotic to hypohydrotic because the persons termed as anhydrotic were not truly devoid of sweat glands.…”

The association of aggressive and chronic periodontitis with systemic manifestations and dental anomalies in a jordanian population: a case control study

“…The development of teeth is believed to be under strict genetic control, which determines the positions, numbers and shapes of different teeth [19]. Furthermore, dental anomalies, such as peg-shaped lateral incisors for example, are well documented components of numerous systemic diseases and syndromes, such as Down's syndrome [17], Witkop tooth and nail syndrome [28], Saethre-Chotzen syndrome [29], submucous cleft palate [30] and Hypohidrotic ectodermal dysplasia [31]. As the genetic basis for various dental anomalies is gradually being revealed [9], it is simultaneously becoming clearer that predisposition to various types of periodontitis is related to genetic polymorphisms in genes encoding certain cytokines and other components of the immune system, such as IL-1 [32] and IL-10 [33].…”

Witkop's syndrome: A case report

“…Typically, toenails are more severely affected than fingernails, and in some instances, nail plates are absent at birth. 4 Hair defects are not specifically associated with Witkop's syndrome, but some patients might have finely textured or diffusely thin hair but neither marked hypotrichosis nor areas of alopecia are seen. 7 Anomalies of facial topography, such as frontal bossing, depressed nasal ridges, and everted lips, commonly seen in patients with x-linked recessive ED, are not seen in Witkop's syndrome.…”

“…Dental anomalies related to hard tissue formation appear to be related to genes coding for structural proteins contributing to the formation of enamel and dentin matrices or to the proteinases capable of degrading these matrices. [3][4][5] Children with conical teeth, hypodontia or anodontia without some other readily apparent etiology should undergo a detailed history and examination to determine the differential diagnosis. Obtaining a family history is important, as Fried's tooth-and-nail syndrome looks very similar to Witkop's toothand-nail syndrome clinically but is inherited in an autosomal recessive fashion.…”

“…It is a rare autosomal dominant disorder characterized by hypodontia and morphological changes in teeth, along with dysgenesis of nails. [4][5][6][7] The congenital absence of teeth can seriously disable the young patient physically and emotionally, especially during adolescence. Retention of primary teeth in the absence of permanent successors is of significance, since they help retain the height of the alveolar bone in an area, which may be a site for further comprehensive therapy.…”