Sweat studies under conditions of moderate heat stress in two patients with the Nägeli-Franceschetti-Jadassohn syndrome.

The sweating response of 2 patients with the Nägeli-Franceschetti-Jadassohn syndrome was measured by direct calorimetry. During the experiment, under conditions of moderate heat stress, the 2 patients felt comfortable and showed a normal thermoregulatory response. This tended to corroborate the patients' history which did not reveal a clear-cut heat intolerance. However, the starch-iodine test on the right flexor forearm 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. Still, the patients' overall performance through the experiment indicated 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 capacity of patients suffering from a hereditary dermatosis in which hypohidrosis is part of the clinical picture.

Involvement of the digestive tract in Cowden's disease.

A 28-year-old white man had Cowden's disease and multiple gastrointestinal polyps. Of the 56 cases of Cowden's disease which have been reported so far, esophageal and gastrointestinal involvement have been observed in only 18 patients. Endoscopic, radiologic, and histopathologic features of these lesions were confirmed in the present patients.