Figure 1.234. The typical facies of hypohidrotic (anhidrotic) ectodermal dysplasia is seen in this infant. Note the alopecia, absent eyebrows and eyelashes, square forehead with frontal bossing, hyperpigmented wrinkles around the eyes, flattened nasal bridge, and large conspicuous nostrils. There are wide cheek bones with depressed cheeks, thick everted lips, a prominent chin, and the ears may be small and pointed. These infants have a thin dry skin, decreased sweating, decreased tearing, and abnormal dentition. The nails are defective in a large percentage of these patients in that they may be thin, brittle, or ridged. If the absence of the sweat glands is generalized, they may have recurrent fever in high environmental temperatures.
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