D. Ayesha Thabusum^1,, N. Rajesh², R. Sudhakara reddy², T. Ramesh²

The Ectodermal Dysplasia comprises a large, heterogeneous group of inherited disorders that are defined by primary defects in the development of two or more tissues derived from embryonic ectoderm. The tissues primarily involved are the skin, hair, nails, eccrine glands and teeth. The X-linked recessive ED (Christ-Siemens-Touraine syndrome) is the most common disorder; it affects males and is inherited through female carriers. It is characterized by the triad of signs comprising 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. Two case reports illustrating the typical features of anhidrotic ectodermal dysplasia have been presented. Since there is no definitive treatment for the disease, an early prompt diagnosis and a multidisciplinary approach in planning a treatment can improve the quality of life of the patient.

Christ-Siemens-Touraine syndrome, hypotrichosis, hypodontia, hypohidrosis