PROSTHETIC MANAGEMENT OF CHRIST-SIEMENS- TOURINE SYNDROME – A CASE REPORT

Ectodermal dysplasias (EDs) are a heterogeneous group of disorders characterized by developmental dystrophies of ectodermal structures. The X-linked recessive ED (Christ-Siemens- Touraine syndrome) is the most common disordered (80%of EDs); it affects males and is inherited through female carriers. It is characterized by the triad of signs comprising of sparse hair (atrichosis or hypotrichosis), abnormal or missing teeth (anodontia or hypodontia) and inability to sweat due to lack of sweat glands (anhidrosis or hypohidrosis). The lack of teeth and the special appearance were reported to be major concerns. The incidence in male is estimated at 1 in 100,000 births, the carriers-incidence is probably around 17.3 in 100,000 women. Early dental intervention can improve patient's appearance, thereby minimizing the associated emotional and psychological problem in these patients. Treatment requires team work by medical personal along with dental professionals. This clinical report describes the prosthetic rehabilitation of 24-year-old male patient with multiple missing and malformed teeth along with other classical oral and general manifestation