[Ectodermal anhidrotic dysplasia (author's transl)].
The E. A. D. is a genodermatosis characterised by absence or important diminuation of sweat glands. The study of 3 children of different families allows us to identify the diagnostic elements: typical facial signs, anhidrosis, rarity of teeth "buds", abnormalities of dermatoglyphes. This diagnosis must be established early, to protect the newborn from accidents of overheating caused by perspiration insufficiency. The sweat glands are not affected alone: exocrine glands also, explaining the fragility of the upper respiratory tract in such patients. The teeth abnormalities are important and need replacement by false teeth, carefully done at an early date and continued later on. Genetic investigation is indispensable to discover women who carry the disease, with a high risk of transmission to their children.
D002675:Child, Preschool; D003878:Dermatoglyphics; D004476:Ectodermal Dysplasia / Q000235:genetics / Q000473:pathology*; D005145:Face / Q000002:abnormalities; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D010375:Pedigree; D013545:Sweat Glands / Q000002:abnormalities / Q000473:pathology; D014071:Tooth Abnormalities / Q000473:pathology
Dysplasie ectodermique anhidrotique.