Hereditary Anhidrotic Ectodermal Dysplasia. Presented by Dr. William B. Reed (by invitation) and Dr. S. William Becker.
This 3-year-old boy has had an absence of sweating since birth. He is one of three siblings. His older brother, also with absence of sweating, died at age 3 years of an overwhelming infection. The oldest brother is normal. The child shows extremely blonde hair, fragile and sparse. He has an absence of eyebrows and a saddle nose, with a chronic rhinitis. He wears full dentures. His skin is extremely dry, pale and glossy in texture. Subcutaneous vessels are easily visualized. His nails are normal. He pigments slightly in the sun, however, despite his light coloration.He lives in an air conditioned house with a swimming pool so that during the warm summer months he can regulate his temperature. The mother has observed some sweating of the feet.His delivery was normal, and