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肾源性纤维化硬皮病 被引量:1

Nephrogenic Fibrosing Dermopathy
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摘要 肾源性纤维化硬皮病是近年来新发现的一种皮肤病。该病和硬皮病的临床表现很相似,都是以局限性皮肤硬化为主要皮损表现。其组织病理特征:真皮层表达CD34的梭形细胞增殖并向下扩展到皮下脂肪组织,间质黏蛋白和弹性纤维轻度增加并伴弹性组织离解;胶原铁染色可见真皮浅层少量黏蛋白沉积。 Nephrogenic fibrosing dermopathy (NFD) is a newly recognized fibrosing skin condition. Its primary clinical manifestation is localized sclerosis of the skin, which is very similar to scleroderma. It has characteristic histopathological changes. The skin-biopsy specimen shows a proliferation of CD34-expressing dermal fibrocytic spindle-cells which extend into subcutaneous fat, a slight increase of interstitial mucin and elongated elastic fibers with elastolysis. A colloidal iron stain reveals the deposition of a small amount of mucin in the superficial dermis. The mechanism of the disorder is unclear, but it is known to all that it mostly develops in patients with renal insufficiency,maybe this clue can help us to find the correct cause.
出处 《国际皮肤性病学杂志》 2006年第1期38-40,共3页 International Journal of Dermatology and Venereology
关键词 硬皮病局限性 纤维化 肾疾病 Scleroderm, limited Fibrosis Kidney diseases
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参考文献18

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同被引文献6

  • 1朱学骏.王宝玺.等编著.皮肤病学.第2版.北京:北京大学医学出版社,2010,10:1809-1810.
  • 2Sharon R, Maria LH, Richard ET, et al. Cutaneous manifesta- tions of chronic graft-versus-host disease. Biol Blood Marrow Transplant, 2006,12( 11 ) : 1101-1113.
  • 3Cowper SE, Robin HS, Steinberg SM, et al. Scleromyxoedema- like cutaneous diseases in renal-dialysis patients. Lancet,2000, 356 (9234) : 1000-1001.
  • 4Gilman AL, Serody J. Diagnosis and treatment of chronic graft- versus-host disease. Semin Hematol,2006,43(1) :70-80.
  • 5Dumford K, Anderson JC. CT and MRI findings in scleroderma- tous chronic graft vs. host disease. J Clin Imaging 25(2) :138- 140.
  • 6朱学骏,孙建方.主译.皮肤病理学与临床的联系.第3版.北京:北京大学医学出版社,2006.12:254.

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