A case of hypohidrotic ectodermal dysplasia in female, A rare x linked recessive disorder

Ectodermal dysplasia is characterized by the absence or defects of two or more ectodermally derived structures. It is commonly inherited as X-linked recessive disorder. Males are most commonly affected and females are usually carriers. In very rare cases it is inherited as autosomal recessive form and in such cases females can manifest complete syndrome. Anodontia or hypodontia is the most striking dental manifestation. In severe hypodontia, there is lack of alveolar development with consequent protrusion and eversion of the lips. Patients with anhidrotic forms suffer from heat intolerance due to lack of sweat glands. A case of a 8-year-old female child with anhidrotic ectodermal dysplasia with complete anodontia is presented. Dental, oral, and physical features were taken into consideration in diagnosis and treatment for this patient. Clinical management consisted of removable complete dentures.