Ectodermal Dysplasia: Report and Analysis of Eleven South Indian Patients with Review of Literature

Abstract

Ectodermal dysplasia represents a rare syndrome affecting two or more ectodermally derived structures. The condition is thought to occur in approximately 1 in every 100,000 live births. It affects men more frequently and severely, while women being the carriers and heterozygote usually show minor defects. There are more than 150 different variants of ectodermal dysplasia (ED) reported in the literature. Most commonly encountered among them is hypohidrotic ED which frequently exhibits the most severe dental anomalies like hypodontia or anodontia along with hypohidrosis and hypotrichosis. Here we make an attempt to collectively report and discuss eleven South Indian patients who reported to our department during the year 1998 to 2004. An added emphasis is laid on family history of consanguineous marriage among the parents of these patients.

Keywords

• Hypohidrotic ectodermal dysplasia
• hypodontia
• hypohidrosis
• hypotrichosis
• anhidrotic
• consanguineous marriage.