Anhidrotic ectodermal dysplasia with palmoplantar keratoderma: an unusual presentation

Abstract

Anhidrotic ectodermal dysplasia (AED), or Christ-Siemens-Touraine syndrome, was first described in 1848 by Thurnam. It is characterized by a partial or complete absence of sweat glands, hypotrichosis and hypodontia. The mode of inheritance is predominantly X-linked but an autosomal recessive pattern has also been observed.(2) Palmoplantar keratoderma is classically a component of hidrotic ectodermal dysplasia; however, we report herein two brothers who had classical manifestations of AED along with palmoplantar keratoderma.