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KMID : 0363219920300020262
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Korean Journal of Dermatology
1992 Volume.30 No. 2 p.262 ~ p.266
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A Case of Focal Dermal Hypoplasia
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Abstract
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Focal dermal hypoplasis (Goltz syndrome) is a rare genetic mesoectoldermal disorder characterized by variable multisystem defects, including anomalies of the skin, skeleton, teeth and eyes.
A 22-year-old female patient presented with confluent and discrete, yellowish nodules on the calves, white linear atrophic lesion. hypo- and hyperpigmented patches. and telangiectasia scattered over the trunk. neck and extremities. In addition to
the
skin lesions. this patient showed hypertrophy of the left leg. anomalies of the left foot. fingers and nails. and maloccluded teeth. Histopathological findings of a yellow nodule revealed accumulations of adipose tissue very near to the epidermis
that
largely replaced the upper dermis.
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KEYWORD
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