Because it is inherited as an X-linked recessive trait, it generally affects only males, but girls who are carriers may also show some signs of the syndrome to greater or lesser degrees.
Affected children are unable to sweat.
This means that they may experience sometimes mysterious episodes of high fever in warm environments, which makes the condition one to be considered during investigation of a case of fever of unknown origin. It is an autosomal dominant hereditary disorder. The teeth are usually normal, although some children have small teeth with weak enamel and numerous caries. Inflammation of the eyes and lids are common. Sweating is always normal.