Congenital ectodermal dysplasia (Hebrew)

Seven ptients aged 2-21 years with various manifestations of congenital ectodermal dysplasia are presented. Two were females, and in both the parents were first cousins. In all, sweating was demonstrated only during severe heat stress. One patient was prevented from completing the sweat test after 40 minutes, as his rectal temperature increased to 40°C. Two other patients complained of fatigue and discomfort. The quantities of sweat produced were smaller than those produced by healthy subjects. These patients are therefore prone to develop heat stroke during hard work in hot climates.