Hereditary Hypohidrotic ectodermal dysplasia: Report of 3 cases with review of literature

Dr. Anjali Kumari; Dr. Prabhat Kumar Singh

Volume : VIII, Issue : VII, July - 2019

Hereditary Hypohidrotic ectodermal dysplasia: Report of 3 cases with review of literature

Dr. Anjali Kumari, Dr. Prabhat Kumar Singh

Abstract :

The ectodermal dysplasias comprise a large, heterogeneous group of inherited disorders that are characterized by primary defects in the development of 2 or more tissues derived from emyonic ectoderm. The two most common types of ectodermal dysplasias are hypohidrotic ectodermal dysplasia (Christ Siemens–Touraine syndrome) and hidrotic ectodermal dysplasia (Clouston syndrome). The tissues primarily involved in hypohidrotic ectodermal dysplasia are skin, hair follicles, eccrine glands, sebaceous glands, nails and teeth. Hypohidrotic hereditary ectodermal dysplasia hence manifests as sparse hair, oligodontia, and reduced sweating. Here we present 3 cases of Hypohidrotic hereditary ectodermal dysplasia of which 2 male patients were siblings and one 6 years old male child patient.