KMID : 0363219950330050961
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Korean Journal of Dermatology 1995 Volume.33 No. 5 p.961 ~ p.966
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A Case of hypohidrotic Ectodermal Dysplasia
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Abstract
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ohidrotic ectodermal dysplasia is a rare and heterogenous genodermatosis that is characterized by hypohidrosis, hypotrichosis, dental hypoplasia, and a characteristic facies. A 16-month-old male was evaluated for anodontia and anhidrosis since
birth.
There was no family history of this disease, His face was characterized by a broad nose and sparse eyebrows and periorbital wrinkling. The skin was dry, and scalp hairs were thin and sparse. Skull X-ray finding revealed nothing remarkable except
delayed
teeth eruption. His developmental status for which we used DDST was slightly reduced on mental development and showed normal on physical development. A skin biopsy was done on skin from the left palm, which showed a few immature eccrine glands in
the
dermis.
We report a typical form of hypohidrotic ectodermal dysplasia with a review of the literature. (Kor J Dermatol 1995;33(5) : 961~966)
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