Familial and Congenital Simple Anhidrosis

THE TERM anhidrotic ectodermal dysplasia originally suggested by Weech in 1929,1is now generally accepted for a syndrome characterized by anhidrosis, hypodontia, and hypotrichosis. This is a congenital disorder of world-wide distribution and although it is rare, the literature that has accumulated on the subject is considerable. Thorough reviews, covering the historical, clinical, and pathological aspects, include those of De Silva,2Upshaw and Montgomery,3Mohler,4and Jespersen.5 In addition to the triad of signs and symptoms mentioned, there are often abnormalities of the skeletal system, facies and nails,6lacrimal glands, mentation and swallowing,2gonads,4mammary glands,7and other systems.8,9 Upshaw and Montgomery in 19493state that "Although this triad of symptoms, namely, hypodontia, hypotrichosis, and anhidrosis has been observed many times during the past hundred years, the literature we have read includes no observation of universal anhidrosis that