Ectodermal dysplasia is a hereditary disorder associated with abnormal development of embryonic ectodermally-derived organs including teeth, nails, hair and sweat glands. Hypodontia of the primary and permanent dentition is the most com-mon oral finding. Therefore, affected patients need dental prosthetic treatments during their developmental years. This re-port presents two cases of children affected by ectodermal dysplasia with anodontia. Oral rehabilitation was accomplished with removable acrylic prostheses. Treatment had major impacts on self-esteem, masticatory function, speech and facial esthetic.
Introduction
Hereditary ectodermal dysplasia represents a large group of conditions in which two or more ectodermally-derived anatomic structures fail to develop. Patients with ectodermal dysplasia are characterized by hypoplasia or aplasia of structures such as skin, hair, nails, teeth, nerve cells, sweat glands, parts of the eye and ear and other organs. Ectodermal dysplasia might be inherited in any form of several genetic patterns including autosomal-dominant, autosomal-recessive, and X-linked modes.2 Although more than 170 different subtypes of ectodermal dysplasia have been identified, these disorders are considered to be relatively rare with an estimated incidence of 1 case per 100,000.
According to the state of sweat glands involvement, two major groups are distinguished: (1) Hypohidrotic or anhydrotic (Christ-Siemens-Touriane syndrome) in which sweat glands are either absent or significantly reduced in number; (2) Hydrotic (Cloustone syndrome) in which sweat glands are normal. Dentition and hair are involved similarly in both types but hereditary patterns of nails and sweat glands involvement are different. Hypohidrotic ectodermal dysplasia as the most common type seems to show an X-linked inheritance pattern with the gene mapping to Xq12-q13; therefore, males are more susceptible than females. Hydrotic type is inherited in an autosomal dominant pattern.6 In general, the skin of affected children is lightly pigmented and appears thin and almost transparent; surface blood vessels are easily visible. Other manifestations include fine sparse hair, reduced density of eyebrow and eyelash hair. When hair is present, it may be fragile, dry, and generally with unruly appearance as a result of poorly developed or absent sebaceous glands. Fingernails and toenails may also show faulty development and be small, thick or thin, brittle, discolored, cracked, and/or ridged.
The pre-ocular skin may show fine wrinkling with hyper-pigmentation and midface hypoplasia is frequently observed resulting in protuberant lips. In cases where the salivary glands are hypoplastic or absent, varying degrees of xerostomia are expected. Affected individuals typically display heat intolerance because of reduced number of eccrine sweat glands. These glands may be either absent, reduced in number, or nonfunctioning (hypohydropic), which may result in elevated body temperature. Fever with unknown origin may lead to early diagnosis during infancy.
The teeth are markedly reduced in number (oligodontia or hypodontia) and often manifest abnormal development in shape which may appear tapered, conical or pointed in incisors. Molars might be observed in reduced size.10 The lack of tooth bud formation causes hypoplastic alveolar bone, leading to a reduced vertical dimension of occlusion. Therefore, an old-age appearance is common in affected individuals.
This article presents the early prosthetic rehabilitation for two children with hereditary ectodermal dysplasia associated with severe oligodontia in primary and mixed dentition.
Authors: Somayeh Hekmatfar, Karim Jafari, Raziyeh Meshki, and Samaneh Badakhsh
Source: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3442425/
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