Lipoid proteinosis in the eastern Mediterranean region of Turkey
作者: Asena C DogramaciMurat M CelikEbru CelikHanifi Bayarogullari
刊名: Indian Journal of Dermatology, Venereology and Leprology, 2012, Vol.78 (3), pp.318-322
来源数据库: Directory of Open Access Journals
DOI: 10.4103/0378-6323.95447
关键词: Consanguineous marriagesGenodermatosisLipoid proteinosisTurkey
原始语种摘要: Background: Lipoid proteinosis (LP), also known as hyalinosis cutis et mucosae or Urbach-Wiethe disease, is a rare autosomal recessive genodermatosis. Hyalin-like material is deposited in multiple organs, including the skin, oral mucosa, larynx, and brain. Only about 300 cases have been reported in the literature so far. Occurrence of LP in siblings is also rare. The reasons for relatively large number of cases, the clinical features of the patients, and the association of LP with other clinical conditions are described in this article. Aims: The aim of this study is to show that LP is not rare in Turkey and consanguineous marriage is still a social problem. Methods: We included patients between the years of 2008-2011 with lesions resembling LP. Based on the findings of clinical and...
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  •  出席的
  •  因此
  • proteinosis 蛋白沉积
  • genodermatosis 遗传性皮肤病
  • consanguineous 同源岩浆的
  • hyalinosis 透娩性
  • brothers 弟兄
  • present 出席的
  • family 出席的
  • therefore 因此
  • eight 因此
  • features 特征