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ectodermal dysplasia, hypohidrotic
Hypohidrotic (anhidrotic) ectodermal dysplasia is a rare, genetically determined condition consisting of three elements: partial or complete absence of sweat glands, abnormal teeth, and sparse hair. 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.
Hidrotic ectodermal dysplasia (Clouston syndrome) is a variant condition, characterized by malformed or absent nails, sparse hair, and thickened
palms and soles. 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. Some patients exhibit absence of eyebrows and lashes, and dark pigmentation over the knees, elbows, and knuckles.