Early Management of Hypohidrotic Ectodermal Dysplasia: Conventional Removable Prosthesis.
Journal Title: International Journal of Medical Science and Innovative Research (IJMSIR) - Year 2019, Vol 4, Issue 4
Abstract
Introduction Hypohidrotic ectodermal dysplasia (HED) is a genetic disease, the development of ectoderm characterized by malformations of ectodermal structures such as skin, hair, teeth and sweat glands. (2, 17 , 23) It is composed of three clinically indistinguishable subtypes associated with impaired sweating as a major symptom: X-linked DEH, autosomal recessive HED (AR-HED) and autosomal dominant HED( AD-HED), as well as a rare fourth subtype with immunodeficiency as the main manifestation (HEDwith immunodeficiency). Its incidence is estimated at 7/10,000 births. (2, 7,19, 23) HED is clinically characterized by oligodontia (absence of at least six permanent teeth), dental morphology abnormalities, craniofacial dysmorphism and alveolar bone hypoplasia, associated with other ectodermal derivative defects. (1, 5, 18) Children with ectodermal dysplasia have characteristic facial abnormalities that can look like old people. The ectodermal anhydrous dysplasia (EAD) or X-linked ectodermal dysplasia (XED), is the most frequent of the ED because it represents 80% of the ED (incidence estimated at 1 / 100,000 births). In this work, we recall the characteristics of HED and specify, through a clinical case of prosthetic rehabilitation, the interest of treatment with conventional prostheses.
Authors and Affiliations
F. Nouara
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