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KMID : 0032220090210010049
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Annals of Dermatology
2009 Volume.21 No. 1 p.49 ~ p.52
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A Case of Non-Hallopeau-Siemens Recessive Dystrophic Epidermolysis Bullosa
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Kang Gyo-Shin
Suh Moo-Kyu
Kim Ae-Suk
Choi Sung-Min
Kim Jae-Hong
Ko Woo-Tae
Kim Dong-Hoon
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Abstract
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Dystrophic epidermolysis bullosa (DEB) is a rare group of heritable mechanobullous disorders that are characterized by blistering and scarring of the skin and mucosae and these lesions are induced by minor trauma, DEB is also associated with nail dystrophy. DEB can be inherited either in an autosomal recessive or dominant fashion. Regardless of the mode of inheritance, DEB is caused by defects of the ultrastructural entity known as the anchoring fibril, which results in separation of the sublamina densa. Recessive DEB (RDEB) is classified into Hallopeau-Siemens and non- Hallopeau-Siemens. We herein report on a case of non- Hallopeau-Siemens RDEB and there was no family history of this malady, and we present the clinical, histological and electron microscopy findings.
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KEYWORD
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Dystrophic epidermolysis bullosa, Non-Hallopeau-Siemens
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