Open AccessHypohidrotic Ectodermal Dyplasia: A Case Series
Author(s) -
Manjari Kishore,
Panat,
Anju Aggarwal,
Nidheesh Agarwal,
Nisha Upadhyay,
K Ajai,
Abhijeet Alok
Publication year - 2014
Publication title -
journal of clinical and diagnostic research
Language(s) - Uncategorized
Resource type - Journals
eISSN - 2249-782X
pISSN - 0973-709X
DOI - 10.7860/jcdr/2014/6597.3951
Subject(s) - hypohidrotic ectodermal dysplasia , hypodontia , medicine , ectodermal dysplasia , family history , dentistry , medical history , dermatology , orthodontics , pediatrics , surgery
ECTODERMAL DYSPLASIA (ED) IS A RARE DISORDER WITH DEFECTS IN TWO OR MORE OF THE FOLLOWING STRUCTURES: the teeth and the 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 syndrome 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 dentally with conventional adult appearing prosthesis which are focused only on the oral manifestations of the syndrome. We are here reporting two classical cases of hypohidrotic ED with a review of the literature.