AIM This paper describes a clinical case of a male with hypohidrotic ectodermal dysplasia submitted to rehabilitation and occlusal dental interventions with follow-up from 3 to 14 years of age. BACKGROUND Due to the severe effects on function and esthetics, the clinical manifestations of ectodermal dysplasia exert a negative impact on quality of life. However, oral rehabilitation in childhood...

Ectodermal dysplasia is a rare hereditary disorder. Its Hypohidrotic (HED) variant is also known as ChirstSiemens-Touraine syndrome. It is inherited as an Xlinked trait. Such Patients are characterized by the clinical manifestations of Hypodontia, Hypotrichosis, Hypohidrosis and a highly characteristic facial physiognomy. This article, reports a typical case of Hypohidrotic Ectodermal Dysplasia...

We report a case of a lower lid ectropion with ectodermal dysplasia and ectropion blepharoplasty surgery experience. A 14-year-old Han nationality male patient with typical characteristics of hypohidrotic ectodermal dysplasia presented to our clinic for his right lower lid eversion. The patient was diagnosed as having hypohidrotic ectodermal dysplasia and underwent an uneventful blepharoplasty ...

We report an inbred Tunisian family, in which the proband manifested signs of hypohidrotic ectodermal dysplasia, subtotal amelia, scoliosis and left renal agenesis. Two other family members had the full clinical criteria of hypohidrotic ectodermal dysplasia, characterized by deficient sweat glands, hypodontia, hypoplasia of the mucous glands, and fine hair. Nine family subjects had variable cli...

Abstract Background Hypohidrotic Ectodermal Dysplasia (HED) is a genetic disorder which affects structures of ectodermal origin. X-linked hypohidrotic dysplasia (XLHED) the most common form disease. XLHED characterized by hypotrichosis, hypohydrosis and hypodontia. The cardinal features classic HED become obvious during childhood. Identification hemizygous EDA pathogenic variant in an affected ...

Lecturer Professor and HOD Dept. of Oral and Maxillofacial Pathology Abstract: D.A.Pandu Memorial R.V.Dental College and Hospital, Banglore. Hypohidrotic ectodermal dysplasia is an uncommon disorder of tissues derived from ectoderm, characterized by the triad of hypohidrosis and hypodontia, which form the essential features of the syndrome 1. A case of a 7 year old child with hypohidrotic ectod...

Ectodermal dysplasia is a rare entity with incidence of 1 in 1,00,000 births male predominance. Most commonly it presents appendageal abnormality facial dysmorphism. The two most common types ectodermal dysplasias are hypohidrotic (Christ-Siemens-Touraine syndrome) and hidrotic (Clouston syndrome). Clinical recognition varies depending on severity symptoms associated complications. prognosis go...

Hypohidrotic ectodermal dysplasia has been mapped to Xq11-q13 by linkage studies and by a translocation in a manifesting female. We report a family with hypohidrotic ectodermal dysplasia in which the disease did not segregate with this region of the X chromosome as expected. Ten DNA probes which are localised between Xp11 and Xq22 were used in the investigation. The difficulties in diagnosing t...

hypohidrotic ectodermal dysplasia (hed) is a rare congenital disorder arising from deficient development of ectoderm-derived structures including skin, nails, glands and teeth. the phenotype of hed is associated with mutation in eda, edar, edaradd and nemo genes, all of them disruptingnf-κb signaling cascade necessary for initiation, formation and differentiation in the embryo and adult. here w...