What is Hypohidrotic ectodermal dysplasia
Ectodermal dysplasia is a hereditary disorder.Hypohidrotic ectodermal dysplasia is a rare congenital disease that affects several ectodermal structures.Before birth, these disorders result in the abnormal development of structures including the skin, hair, nails, teeth, and sweat glands.Hypohidrotic ectodermal dysplasia is a rare genetic condition that can result from mutations in one of several genes (these include ectodysplasin a (eda), eda receptor (edar), edaradd and wnt ioa).A reduced ability to sweat (hypohidrosis) missing teeth, (hypodontia) and.
Ectodermal dysplasia is a large group of inherited disorders characterised by a primary defect in hair, teeth, nails or sweat gland function, in addition to another abnormality in any tissue of ectodermal origin.Hed is the most common of the ectodermal dysplasias and the characteristic clinical feature of individuals affected with hed is hypohidrosis (reduced ability to sweat) with subsequent impaired thermoregulation.The diagnostic tool is the typical clinical physionomy.Common symptoms include sparse scalp and body hair, reduced ability to sweat, and missing teeth.Hypohidrotic ectodermal dysplasia is the most common form of ectodermal dysplasia.
More than 180 specific types of this condition have been identified.Manifestations of the disease differ in severity and may involve teeth, skin, hair, nails, and sweat.Hed is primarily characterized by partial or complete absence of certain sweat glands (eccrine glands.Common symptoms include sparse scalp and body hair, reduced ability to sweat, and missing teeth.Some individuals with ed may also have cleft lip and/or palate.