Boys with the genetic condition ectodermal dysplasia. Two young brothers aged 4 years (left) and 2 1/2 years, both of whom have X-linked hypohidrotic


Boys with the genetic condition ectodermal dysplasia. Two young brothers aged 4 years (left) and 2 1/2 years, both of whom have X-linked hypohidrotic ectodermal dysplasia (HED), seen with their mother and sister (six months). The mother comes from a long line of carriers of ED. Ectodermal dysplasia (ED) describes a group of developmental conditions affecting the ectoderm, the layer of cells that form the skin and nervous system. Typical symptoms include very fine hair, thin skin, missing or mis-shapen teeth and high susceptibility to eczema and asthma. In HED, there is the added complication that the sufferer has a reduced capacity to sweat. As sweating is the main way the body loses heat, particular care must be taken to prevent hyperthermia, especially in the summer. X-linked HED results from a mutaion of the EDA gene on the X chromosome.


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Keywords: -linked, age, autosomal, boy, boys, brothers, children, chromosome, condition, development, developmental, dysplasia, ectodermal, ed, family, genetic, happy, hed, hypohidrotic, inherited, male, mother, mutation, recessive, year, years, young