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类脂蛋白沉积症1例 被引量:3

Lipoid Proteinosis:one case report
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摘要 报告1例类脂蛋白沉积症。14岁男性,头面、四肢、腰部反复发生红斑、水疱、疤痕,伴声音嘶哑12年。组织病理检查可见真皮乳头层血管壁和汗腺周围有透明蛋白样物质沉积,PAS染色强阳性。 A 14-year-old boy presented with a complaint of hoarsenes as well as eryth ema,blisters and scars lesions on th e face,limbs and waist in the last 12years is reported.The histopathological examination of the skin specimens showed that deposits of homogeneous hyaline -like material,strong positive on periodic acid-schiff staini ng,were found around the dermal capi llaries and sweat glands.
出处 《临床皮肤科杂志》 CAS CSCD 北大核心 2002年第1期43-44,共2页 Journal of Clinical Dermatology
关键词 类脂蛋白沉积症 病理 诊断 治疗 lipoid proteinosis
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参考文献5

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同被引文献17

  • 1Acar A, Eryilmaz A, Gocer C, et al. Lipoid proteinosis of larynx: review of four cases [J]. Int J Pediatr Otorhinolaryngol,2004, 68(12): 1557-1561.
  • 2Ringpfeil F. Selected disorders of connective tissue: pseudoxanthoma elasticum, cutis laxa,and lipoid proteinosis[J]. Clin Dermatol, 2005, 23(1): 41--46.
  • 3Hamada T, McLean WH, Ramsay M, et al. Lipoid proteinosis maps to 1q21 and is caused by mutations in the extracellular matrix protein 1 gene (ECM1)[J]. Hum Mol Genet, 2002, 11(7): 833-840.
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  • 5Fujimoto N, Terlizzi J, Aho S, et al. Extracellular matrix protein 1 inhibits the activity of matrix metalloproteinase 9 through high-affinity protein/protein interactions[J]. Exp Dermatol, 2006, 15(4): 300-307.
  • 6Chan I, South AP, McGrath JA, et al. Rapid diagnosis of lipoid proteinosis using an anti-extracellular matrix protein 1 (ECM1) antibody[J]. J Dermatol Sci, 2004, 35(2): 151-153.
  • 7Van Hougenhouck - Tulleken W, Chan 1, Hanaada T, et al. Clinical and molecular characterization of lipoid proteinosis in Namaqualand,South Africa. Br J Dermatol 2004; 151:413 - 423.
  • 8Hamada T, Mclean WH, Ramsay M, et al. Lipoid proteinosis maps to lq21 and is caused by mutations in the extracelltdar matrix omtein 1 gene ( ECM 1 ). Hum Mol Genet 2002 ; 11 : 833 - 840.
  • 9Hamada T, Wessagowit V, South AP, et al. Extracellular matrix protein 1 gene (ECMI) mutations in lipoid proteinosis and genotype- phenotype correlation. J Invest Dermatol 2003 ; 120: 345 - 350.
  • 10李新.类脂蛋白沉着症1例[J].中华皮肤科杂志,1994,27(3):181-181.

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