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“颗粒状”弹性纤维。青霉胺长期给药的一个标志物。

The "lumpy-bumpy" elastic fiber. A marker for long-term administration of penicillamine.

作者信息

Gebhart W, Bardach H

出版信息

Am J Dermatopathol. 1981 Spring;3(1):33-9.

PMID:7235197
Abstract

A 29-year-old woman with Wilson's disease developed dermolytic skin lesions 2 years after initiation of treatment with penicillamine. Eight years later, still on penicillamine therapy, striae appeared over both of her breasts. Biopsy of involved skin during the 10th year of treatment with penicillamine revealed characteristic lumpy-bumpy alterations of dermal elastic fibers which were not present in the first skin biopsy 8 years previously. Biopsy of a stria showed changes similar to those in the dermolytic skin lesions. Lumpy-bumpy elastic fibers are pathognomonic for penicillamine-induced elastosis. They are easily recognizable with examination by conventional microscopy. Their appearance may serve as a warning of potentially serious, widespread elastic tissue involvement. These abnormal elastic fibers are not only found in the skin, but also in the lungs.

摘要

一名患有威尔逊氏病的29岁女性在开始使用青霉胺治疗2年后出现了皮肤溶解性病变。8年后,她仍在接受青霉胺治疗,双侧乳房出现了萎缩纹。在使用青霉胺治疗的第10年,对受累皮肤进行活检发现真皮弹性纤维有特征性的粗颗粒状改变,而8年前的首次皮肤活检中并未出现这种改变。对一条萎缩纹的活检显示出与皮肤溶解性病变相似的变化。粗颗粒状弹性纤维是青霉胺诱导的弹性组织变性的特征性表现。通过传统显微镜检查很容易识别它们。它们的出现可能预示着潜在的严重、广泛的弹性组织受累。这些异常弹性纤维不仅见于皮肤,也见于肺部。

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