SWEAT STUDIES UNDER CONDITIONS OF MODERATE HEAT-STRESS IN 2 PATIENTS WITH THE NAGELI-FRANCESCHETTI-JADASSOHN SYNDROME

The sweating response of 2 patients with the Nageli-Franceschetti-Jada ssohn syndrome was measured by direct calorimetry. During the experime nt, under conditions of moderate heat stress, the 2 patients felt comf ortable and showed a normal thermoregulatory response. This tended to corroborate the patients' history which did not reveal a clear-cut hea t intolerance. However, the starchiodine test on the right flexor fore arm revealed a marked reduction in activated sweat gland density while the patients were still under experimental conditions. Probably this was not the only region with a reduced number of functioning glands. S till, the patients' overall performance through the experiment indicat ed that their total number of functioning glands was able to produce a sweat output sufficient to prevent disturbances in thermoregulation. To our knowledge, this is the first study of the thermoregulatory capa city of patients suffering from a hereditary dermatosis in which hypoh idrosis is part of the clinical picture.