Direct Composite Resin Application, and Prosthetic Management in a Patient with Hypohidrotic Ectodermal Dysplasia: a Case Report

Ectodermal dysplasia is a rare group of disorders affecting the nails, hair, teeth, and sweat glands to a variable degree. The most common form of ectodermal dysplasia is X-linked hypohidrotic ectodermal dysplasia, which affects males more severely, while heterozygous females exhibit variable severity, ranging from mild to severe, because of inactivation of the X-chromosome. The 7-year, 3-month-old boy presented the classical features of hypohidrotic ectodermal dysplasia, including diffusely sparse hair, eyelashes and eyebrows, severe hypohidrosis, and subsequent problems with thermoregulation, dry skin and fingernail defects. Considering the clinical situation, the age and potential growth of the young patient, a maxillary RPD and mandibular complete denture were determined to be the treatment of choice. HED is usually a difficult condition to manage prosthodontically, because of the typical oral deficiencies and afflicted individuals are quite young to receive extensive prosthodontic treatment, which restores their appearance, for the development of a positive self-image. (Journal of International Dental and Medical Research 2009; 2: (1), pp. 19-24 )