Coexistence of pseudoxanthoma elasticum-like papillary dermal elastolysis and linear focal dermal elastosis.
Coexistence of pseudoxanthoma elasticum-like papillary dermal elastolysis and linear focal dermal elastosis.
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弹性纤维假黄瘤样乳头状真皮弹性组织溶解和线状局灶性真皮弹性组织增生并存。
DOI：
10.1067/mjd.2002.108495
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发表时间：
2002
影响因子：
13.8
通讯作者：
A. Ishibashi
中科院分区：
文献类型：
--
作者：
A. Akagi;S. Tajima;A. Kawada;A. Ishibashi
一位89岁的日本女性，颈部有弹性假黄瘤样乳头状真皮弹力溶解，腿部和大腿屈肌部位有线状局灶性弹力纤维增生症。两种疾病的病变具有相似的组织学类型-乳头状真皮中弹性纤维的丢失，乳头状下或中层真皮中看起来正常的弹性纤维的聚集。免疫组织化学分析显示，在两种病变的乳头状真皮中，弹性蛋白和微原纤维相关蛋白(纤维蛋白1和2以及微原纤维相关糖蛋白1和4)缺失或减少，并存在于积累的弹性纤维中。同一患者不同部位的两种疾病之间的联系以及疾病之间的组织学和免疫组织学相似性表明，尽管临床上存在差异，但这两种疾病是密切相关的。
An 89-year-old Japanese woman had pseudoxanthoma elasticum-like papillary dermal elastolysis on the neck and linear focal elastosis on the flexor aspects of the legs and thighs. The lesions of both diseases had similar histologic patterns-loss of elastic fibers in the papillary dermis and accumulation of normal-appearing elastic fibers in the subpapillary or mid dermis. Immunohistochemical analysis showed elastin, and microfibril-associated proteins (fibrillin 1 and 2 and microfibril associated glycoprotein 1 and 4) were absent or decreased in the papillary dermis and present in the accumulated elastic fibers in both lesions. The association of 2 diseases on different areas of the same patient and histologic and immunohistologic similarities between the diseases suggest that, despite the clinical differences, both diseases are closely related.
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：
BRAVERMAN, IM;FONFERKO, E
通讯作者：
FONFERKO, E