Hypohidrotic Ectodermal Dysplasia : A Report of Two Cases in a Family

Abstract

Ectodermal Dysplasia (ED) is a rare disorder with defects in two or more of the following structures: the teeth, skin and its appendages including hair, nails, eccrine, and sebaceous glands. Dental manifestations include hypodontia, complete anodontia or malformed teeth. The most common form of the ED is hypohidrotic ED and is usually inherited as an X-linked recessive trait. Female carriers may have a variable degree of clinical manifestations. The condition is thought to occur in approximately 1 in every 100, 000 live births. Dental treatment for these patients varies on an individual basis. Children with ED are often treated for dental problems with conventional adult appearing prosthesis. Here with we are reporting two classical cases of hypohidrotic ED with a review of the literature.