Familial generalized anhidrosis.
A young Caucasian male, otherwise healthy, had had generalized anhidrosis since early childhood. During heat stress tests (40 degrees C, 40% relative humidity), he was found to be heat intolerant since sweat was not apparent. His reaction to muscarinic stimulation of sweat glands was 10% of normal. On biopsy, the sweat glands were morphologically intact, and function of his cardiovascular autonomic responses was normal. The patient's mother reported reduced sweating and her response to muscarinic stimulation was 50% of normal, but his father and both sisters sweated normally. The data suggest a post-ganglionic defect, which may be genetic. To our knowledge this is the first reported case of familial generalized anhidrosis without anatomopathological lesions affecting sweat glands.
Dann
E J
EJ
Heller Institute of Medical Research, Chaim Sheba Medical Center, Tel-Hashomer, Israel.
Epstein
Y
Y
Sohar
E
E
D000293:Adolescent; D000328:Adult; D001833:Body Temperature Regulation; D005192:Family Health; D005260:Female; D006358:Hot Temperature; D006801:Humans; D007007:Hypohidrosis / Q000151:congenital / Q000235:genetics* / Q000503:physiopathology; D008297:Male; D013546:Sweating