Ectodermal Dysplasia

Ectodermal dysplasia (ED) is a heterogenous group of genetic syndromes all deriving from abnormalities ofthe ectodermal structures, showing hypoplasia or aplasia of skin, teeth, appendageal structures (hair, nail, sweat and sebaceous glands), eyes, ears or/and other organs depending on the particular syndrome. There are two major groups of ED including; anhidrotic or hypohidrotic ED, in which sweat glands are either absent or significantly reduced in number and hidrotic ED with normal sweat glands. In addition, there are some recognized ED-like syndromes such as ankyloblepharon-ectodermal dysplasia-clefting syndrome (Hay-Wells syndrome), Rapp-Hodgkin syndrome, Witkop’s syndrome (tooth-and-nail syndrome), etc...