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. ...

Ectodermal dysplasia (ED) is a rare group of inherited disorders with defects in the development one or more tissues derived from primary embryonic ectodermal derivatives. The inclusion ED does not include all derivatives and manifested defect skin, hair, teeth, eccrine glands. Dry frontal bossing, scanty eyebrows, hair follicles are common features seen these patients. patient may suffer high ...

Ectodermal dysplasia is a rare hereditary disorder with a characteristic physiognomy. It is a genetic disorder affecting the development or function of the teeth, hair, nails and sweat glands. Depending on the particular syndrome ectodermal dysplasia can also affect the skin, the lens or retina of the eye, parts of the inner ear, the development of fingers and toes, the nerves and other parts o...

Virender Kumar, Lalit Kumar Dr HSJ Institute of Dental Sciences and Hospital Panjab University Chandigarh, India Ectodermal dysplasia (ED) is a hereditary disease characterized by anomalies in the structures of ectodermal origin. The disease affects skin, saliva, sebaceous and sweat glands (anhidrosis or hypohidrosis), hair (atrichosis or hypotrichosis), nail and teeth (anodontia or hypodontia)...

Recently, patients with hypohidrotic/anhidrotic ectodermal dysplasia (H/AED) have been reported to have a higher prevalence of symptoms suggestive of atopic disorders than the general population. To better understand atopic diathesis in H/AED, 6 cases of clinically or genetically diagnosed H/AED were examined. The following criteria were evaluated with patient consent: sweating, blood test resu...

Hypohidrotic ectodermal dysplasia (HED) is characterized by severe hypohidrosis, hypoplasia of sweat, sebaceous, submucous, meibomian and mammary glands, hypotrichosis, and oligodontia. In early childhood, HED is a life-threatening disorder based on the risks for hyperthermia and pneumonia. Awareness of the hazards related to this rare genetic disease is most helpful in preventing avoidable cal...

A case presented here is that of a nine year old male patient with total anodontia. Findings of this case as regards to the orofacial, radiographic and other general manifestations were suggestive of hypohidrotic ectodermal dysplasia. The dental problems were best managed by prosthetic replacement of dentition taking into consideration a design which would provide adequate relief for the preser...