Hypohidrotic Ectodermal Dysplasia: A Casereport and Literature Review

Abstract

Ectodermal dysplasias (EDs) are a heterogeneous group of disorders characterized by developmental dystrophies of ectodermal structures. Hypohidrotic ectodermal dysplasia is a congenital syndrome, characterized by hypotrichosis (hair is sparse, fine and weak; anomalies in the skin and nails), hypohidrosis (due to the paucity of sweat glands which in turn gives rise to sweat disorders) and hypodontia (partial, and occasionally total, absence of primary and/or permanent dentition). Most patients with EDA have a normal life expectancy and normal intelligence. However, the lack of sweat glands may lead to hyperthermia, followed by brain damage or death in early infancy, if unrecognized. Thus an early diagnosis is important. A case report of ectodermal dysplasia has been reported with review of literature.