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原发性皮肤淀粉样变研究进展 被引量：3

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摘要 原发性皮肤淀粉样变（PCA）是一种多病因的综合征，主要表现为真皮乳头层大量的淀粉样蛋白沉积。本病属常染色体显性遗传，可能存在遗传异质性。最新的遗传学研究将其致病基因定位于染色体lq23和染色体5p13．1-q11．2两个区域。临床上主要分为丘疹型（苔藓样）、斑疹型、结节型（肿胀型）和家族性原发性限局性皮肤淀粉样变。皮损好发于两小腿胫前、臂外侧、背部、大腿、躯干和颈部。典型的临床表现为针头大小的褐色班点，渐变为针头样丘疹，以后发展为苔藓状或结节状或皮肤异色，可伴有剧烈瘙痒。本文就其遗传机制、临床表现、诊断及治疗等做一综述。
作者 袁锋(综述) 王培光(审校) 杨森(审校) 张学军(审校)
机构地区 安徽医科大学皮肤病研究所、安徽医科大学第一附属医院皮呋性病科
出处 《中国麻风皮肤病杂志》 2008年第12期981-983,共3页 China Journal of Leprosy and Skin Diseases
关键词 原发性皮肤淀粉样变 淀粉样蛋白 发病机制
分类号 R597.2 [医药卫生—内科学] R596.1 [医药卫生—内科学]
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参考文献20

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同被引文献16

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  • 9Lee DD,Huang JY,Wong CK,et al.Genetic heterogeneity of familial primary cutaneous amyloidosis:lack of evidence for linkagwith the chromosome 10 pericentromeric region in Chinese families[J].J Invest Dermatol,1996,107(1):30-33.
  • 10Verga U,Fugazzola L,Cambiaghi S,et al.Frequent associationbetween MEN2A and cutaneous lichen amyloidosis[J].Clin Endocrinol,2003,59(2):156-161.

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中国麻风皮肤病杂志
2008年 第12期

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