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KMID : 0363220130510010032
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Korean Journal of Dermatology
2013 Volume.51 No. 1 p.32 ~ p.35
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Epidermal Nevus Syndrome Associated with Ipsilateral Monostotic Fibrous Dysplasia: An Uncommon Skeletal Abnormality
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Lee Hyun-Joo
Lee Seung-Jae
Park Ji-Hye
Choi Chong-Won
Lee Ga-Young
Kim Won-Serk
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Abstract
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Epidermal nevus syndromes are a group of congenital syndromes comprising epidermal nevi in conjunction with the central nervous system, ocular, musculoskeletal, and other organ anomalies. Skeletal manifestations in epidermal nevus syndromes are common, ranging from 50% to 66% of patients. However, fibrous dysplasia of the bone has been rarely described in association with epidermal nevus syndromes. Herein, we report a 46-year-old-male patient who presented with extensive epidermal nevi associated with monostotic fibrous dysplasia of the rib which is located ipsilateral to the skin findings.
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KEYWORD
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Epidermal nevus syndrome, Fibrous dysplasia
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