Anhidrotic ectodermal dysplasia

1 - INTRODUCTION

The ectodermal dysplasias form a heterogeneous group of over 200 rare diseases characterised by the involvement of at least two of the following tissues derived from embryonic ectoderm: teeth, nails, sweat glands, and hair. The incidence of these conditions, all forms combined, is estimated at 7 per 10,000 births. Although most of these forms combine abnormalities from only ectodermal tissue derivatives, of which anhidrotic ectodermal dysplasia is the main form, other more complex forms bring together other, more disparate manifestations: ophthalmological signs, cleft lip and palate etc. They are called syndromic forms.