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KMID : 0363220090470040431
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Korean Journal of Dermatology
2009 Volume.47 No. 4 p.431 ~ p.434
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A Case of Pseudoxanthoma Elasticum-Like Papillary Dermal Elastolysis on the Neck of a Young Woman
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Lee Joon-Ho
Kwon Hyok-Bu
Lee Ai-Young
Lee Seung-Ho
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Abstract
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Pseudoxanthoma elasticum-like papillary dermal elastolysis (PXE-like PDE) is a rare acquired elastolytic disorder that clinically resembles pseudoxanthoma elasticum. The pathogenesis of this disease is uncertain, but it seems to be related to intrinsic aging, abnormal elastogenesis, a genetic predisposition and exposure to ultraviolet radiation. It generally develops on the sides of the neck and supraclavicular regions in elderly persons. Histologically, the characteristic features are the loss of elastic fibers in the papillary dermis and no solar elastosis. We report here on a case of PXE-like PDE in a 26-year-old female whose skin lesion developed 9 years previously. Despite the preferential occurrence at an old age, our patient was young.
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KEYWORD
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Elastolytic disorder, Intrinsic aging, Pseudoxanthoma elasticum-like papillary dermal elastolysis
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