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KMID : 0363220070450101083
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Korean Journal of Dermatology
2007 Volume.45 No. 10 p.1083 ~ p.1086
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A Case of Autosomal Recessive Type 2 Pseudoxanthoma Elasticum Presenting Generalized Cutaneous Laxity
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Hwang Seon-Wook
Cho Kyung-Jong
Oh Doo-Jin
Lee Deborah
Kim Jung-Wook
Park Sung-Wook
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Abstract
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Pseudoxanthoma elasticum is a rare, heritable disorder of elastic fibers characterized by yellowish, coalescing papules on the loose and wrinkled flexural skin. An 18-year-old man presented cutis laxa-like marked wrinkling on the axillae, trunk and thighs for about 10 years. There was no similar skin lesion on his family members and no other systemic involvement was found. Histopathological examination of the abdomen revealed fragmentation and calcification of elastic fibers in the middle and lower dermis. Von Kossa staining showed calcium deposition along the altered elastic fibers. Herein, we report a rare case of autosomal recessive pseudoxanthoma elasticum presenting generalized cutaneous laxity.
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KEYWORD
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Autosomal recessive, Pseudoxanthoma Elasticum, Generalized laxity
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