IJSR International Journal of Scientific Research 2277 - 8179 Indian Society for Health and Advanced Research ijsr-8-7-19874 Original Research Paper Hereditary Hypohidrotic ectodermal dysplasia: Report of 3 cases with review of literature Anjali Kumari Dr. July 2019 8 7 01 02 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 embryonic 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.