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KMID : 0363220080460010122
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Korean Journal of Dermatology
2008 Volume.46 No. 1 p.122 ~ p.125
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A Case of Goltz Syndrome
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Bae You-In
Won Young-Ho
Kim Seong-Jin
Lee Jee-Bum
Lee Seung-Chul
Yun Sook-Jung
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Abstract
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Goltz syndrome or focal dermal hypoplasia, is a rare genodermatosis, characterized by multiple abnormalities of mesodemal and ectodermal organs. Cutaneous manifestations include linear or cribriform atrophied patches with telangiectasia following Blaschko line and papillomas. The majority of patients also have skeletal abnormalities, such as syndactyly, oligodactyly, scoliosis, and short stature. Furthermore, various ophthalmic and dental manifestations have been reported. We report a case of Goltz syndrome occurring in a 2-year old female with asymptomatic erythematous atrophic patches with purpuric macules and telangiectasia mainly on extremities. She also had hypotrichia on vertex and a notching-like cleft on upper lip. A skin biopsy performed on her atrophic patch on the leg revealed dermal hypoplasia. The final diagnosis was made by typical cutaneous manifestations and histopathological findings compatible with focal dermal hypoplasia.
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KEYWORD
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Focal dermal hypoplasia, Goltz syndrome
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