Ectodermal dysplasia (ED) syndrome
نویسندگان
چکیده
Ectodermal dysplasia (ED) syndrome comprises a large, heterogeneous group of inherited disorders that are defined by primary defects in the development of 2 or more tissues derived from the embryonic ectoderm. The tissues primarily involved are the skin and its appendages (including hair follicles, eccrine glands, sebaceous glands, nails) and teeth. The clinical features include sparse hair, abnormal or missing teeth, and an inability to sweat due to lack of sweat glands. One such case report of ectodermal dysplasia is presented here.
منابع مشابه
Dental Management of Ectodermal Dysplasia Syndrome at an Early Age: A Case Report
Objectives: Ectodermal dysplasia (ED) is a relatively common sex-linked dermatitis characterized by congenital dysplasia of one or more ectodermal structures and their accessory appendages. Common manifestations include fragile skin and nails, defective teeth and salivary glands, frontal bossing with prominent supra orbital ridges, nasal bridge depression and protuberant lips. Teeth are often f...
متن کاملComplications of Implant Rehabilitation in Ectodermal Dysplasia Patients: A Review Study
Background and Aim: Ectodermal dysplasia (ED) is a hereditary disease that affects ectodermal tissues. Its oral manifestations include hypodontia or oligodontia, which cause the reduction of the height and width of the alveolar ridge. Considering numerous difficulties that these patients encounter with regard to facial appearance, talking, and chewing, their rehabilitation has a major influence...
متن کاملسندرم Ectrodactyly– ectodermal dysplasia clefting: گزارش مورد به همراه علائم دهانی
Objectives: Ectrodactyly-ectodermal dysplasia-clefting syndrome (EEC) is a rare syndrome with features of ectrodactyly, ectodermal dysplasia and cleft lip/palate. This study presents an Iranian case with classic features and oral complications. Case: We report a 20-year old female with all manifestations of this syndrome. The simultaneous presence of these three malformations is extremely r...
متن کاملA First Look: Determinants of Dental Care for Ectodermal Dysplasia Patients
Ectodermal Dysplasia (ED) is a hereditary disorder characterized by abnormal development of certain tissues and structures of ectodermal origin. The most frequently reported ED syndrome is X-linked hypohidrotic dysplasia, also known as Christ-Siemens-Touraine syndrome, which affects one to seven individuals per 10,000 live births. Orofacial characteristics of this syndrome include anodontia or ...
متن کاملChondroectodermal dysplasia (Ellis-Van Creveld syndrome: A case report
Ellis-Van Creveld syndrome is a very rare congenital disorder which its principal features are polysyndactyly, chondrodysplasia, cardiac abnormalities and ectodermal dysplasia. We report a 10-year-old girl with major manifestations of this syndrome who also had multiple brownish macules and patches on trunk and extremities with aortic and pulmonary stenosis in echocardiographic evaluations.
متن کاملEctodermal Dysplasia Showing the Clinical Overlap between Hay Wells Syndrome and Bowen Armstrong Syndrome
BACKGROUND Several clinical entities combine ectodermal dysplasia (ED) and cleft lip and/or palate (CL/P). These disorders have been recognized with a narrow phenotypic spectrum and very similar clinical features. CASE PRESENTATION We report a case with a clinical diagnosis of Hay Wells syndrome (ankyloblepharon, ED and CL/P), who is a descendent of a mother with Bowen Armstrong syndrome (ED,...
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عنوان ژورنال:
دوره 4 شماره
صفحات -
تاریخ انتشار 2014