Abstract
because of the small number of epidermal appendages, or to other causes is not known. Scaling of the skin did not seem to be as consistent a feature of any form of ectodermal dysplasia other than X-L HED, although the numbers are too small to be conclusive. Early diagnosis of X-L HED has several advantages. Life-threatening complications can be prevented, genetic counseling can be given early, and the parents have an opportunity to plan appropriately for the future (e.g., changing the environment to control heat exposure, obtaining and maintaining adequate dental and medical insurance). Scaling or peeling of the skin of a newborn infant, especially a male infant, should alert the examiner to the possibility of X-L HED. Careful, directed examination is warranted and a detailed family history should be obtained. Examination of the mother may be helpful. If suspicion is high, dental radiographs will reveal diagnostic tooth abnormalities. Skin biopsy is usually not necessary for confirmation.
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