Hypohidrotic ectodermal dysplasia – A case series

Hypohidrotic ectodermal dysplasia – A case series

Author: 
Sathvikalakshmi, B.R., Uma Reddy, CH., Kirthika, R., Chandrashekar, Sudarshan and Vigneshwari
Abstract: 

Ectodermal dysplasia (ED) is a rare heterogeneous group of inherited disorder that share primary defects in the development and functioning of two or more tissues derived from ectoderm like skin, hair, nails, exocrine glands and teeth. This disorder is usually congenital, diffuse and non-progressive. Till date more than 192 distinct varieties in this disorder have been recorded and described. ED has 2 major types, on the basis of the number and functional abilityof the sweat glands namely X-linked anhidrotic and/or hypohidrotic, where sweat glands are either absent or significantly reduced in number (Christ-Siemens-Touraine syndrome);and the next one is hidrotic, where sweat glands are normal and the condition is inherited as autosomal dominant (Clouston’ssyndrome). The Hypohidrotic form exhibits the classical traid of hypohidrosis,hypotrichosis and hypodontia.It is most common type accounting for 80% of EDs, which is X-linked recessive. Most commonly affects males and is inherited through a female carrier. In the hidrotic form teeth, hair and nails are affected whereas the sweat glands are usually not affected. In this article we will be seeing a series of 3 cases of ectodermal dysplasia and their prominent features giving us an idea about the disorder.

Paper No: 
1498