April 1967

Familial and Congenital Simple Anhidrosis

Author Affiliations

Shiraz, Iran
From the Department of Neurology, Nemazee Hospital, and Pahlavi University Medical School, Shiraz, Iran. Dr. Mahloudji is now at the Moore Clinic, Johns Hopkins Hospital, Baltimore.

Am J Dis Child. 1967;113(4):477-479. doi:10.1001/archpedi.1967.02090190123015

THE TERM anhidrotic ectodermal dysplasia originally suggested by Weech in 1929,1 is 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,2 Upshaw and Montgomery,3 Mohler,4 and Jespersen.5

In addition to the triad of signs and symptoms mentioned, there are often abnormalities of the skeletal system, facies and nails,6 lacrimal glands, mentation and swallowing,2 gonads,4 mammary glands,7 and other systems.8,9

Upshaw and Montgomery in 19493 state 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

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