Pseudoxanthoma elasticum‐like papillary dermal elastolysis: report of four Japanese cases and an immunohistochemical study of elastin and fibrillin‐1
Pseudoxanthoma elasticum‐like papillary dermal elastolysis: report of four Japanese cases and an immunohistochemical study of elastin and fibrillin‐1
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弹性纤维假黄瘤样乳头状真皮弹性溶解症：四例日本病例报告以及弹性蛋白和原纤维蛋白-1 的免疫组织化学研究
DOI：
10.1046/j.1365-2133.1998.02332.x
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发表时间：
1998
影响因子：
10.3
通讯作者：
Sakamoto
中科院分区：
文献类型：
--
作者：
Ohnishi;Tajima;Ishibashi;Inazumi;Sasaki;Sakamoto
我们报告四例弹性纤维性假黄瘤样乳头状真皮弹性纤维溶解症（PDE）。颈部多发性小丘疹，临床上类似于弹性假黄瘤，并在乳头状真皮弹性纤维网络的损失被发现在每一个案件。1例患者的弹性蛋白和弹力蛋白-1免疫组织化学染色显示乳头状真皮中弹性蛋白和弹力蛋白-1消失。老年人的部位匹配的正常皮肤显示出完整的弹性蛋白，但在乳头状真皮中缺乏弹性蛋白-1。年轻的正常皮肤显示完整的弹性蛋白和胶原蛋白-1。结果表明，老年人正常颈部皮肤的乳头状真皮中不存在Escherin-1，PDE的主要缺陷可能是弹性蛋白而不是Escherin-1。
We report four patients with pseudoxanthoma elasticum‐like papillary dermal elastolysis (PDE). Multiple small papules on the neck, clinically resembling pseudoxanthoma elasticum, and loss of the elastic fibre network in the papillary dermis were found in each case. Immunohistochemical staining for elastin and fibrillin‐1 in one patient demonstrated the disappearance of elastin and fibrillin‐1 in the papillary dermis. Site‐matched normal skins of the elderly showed intact elastin but a lack of fibrillin‐1 in the papillary dermis. The younger normal skins revealed intact elastin and fibrillin‐1. The results suggest that fibrillin‐1 is absent from the papillary dermis of the normal‐appearing neck skin of the elderly and that the primary defect in PDE may be in elastin rather than in fibrillin‐1.
Fibrillin-1 和 fibrillin-2 在发育中的弹性组织中表现出时间和组织特异性的表达调节。
影响因子：
2.9
作者：
Mariencheck,MC;Davis,EC;Zhang,H;Ramirez,F;Rosenbloom,J;Gibson,MA;Parks,WC;Mecham,RP
通讯作者：
Mecham,RP
影响因子：
6.5
作者：
BERNSTEIN, EF;CHEN, YQ;UITTO, J
通讯作者：
UITTO, J