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J Korean Acad Pediatr Dent. 2000;27(2): 222-228.
오소희1, 권순원1, 김종수1, 김용기1, 임헌송2
1단국대학교 치과대학 소아치과학교실
2단국대학교 치과대학 보철학교실
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Ectodermal dysplasia is a hereditary disease characterized by a congenital dysplasia of one or more ectodermal structures and their accessory appendages. At least 120 subtypes of ectodermal dysplasia have been reported. The disease is usually transmitted as an X-linked recessive trait in which the gene is carried by the female and manifested in the male. Hypohidrotic ectodermal dysplasia is manifested as a triad of defects that include hypohidrosis, hypotrichosis and hypodontia. The characteristic facial features consist of asteatosis, onchodysplasia, sparse and fine blond hair, prominent forehead, a depressed nasal bridge, thick everted lips. The patient may suffer from dry skin, hyperthermia and unexplained high fever as a result of the deficiency of sweat glands. This case report presents detailed procedures of rehabilitating functional and esthetic defect of a 6-year-old boy with hypohidrotic ectodermal dysplasia along with the review of relevant literatures.
Keywords: Ectodermal dysplasia | Hypodontia | Complete denture
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