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青霉胺诱发匐行性穿通性弹力纤维病。冰山一角?

Penicillamine-induced elastosis perforans serpiginosa. Tip of the iceberg?

作者信息

Price R G, Prentice R S

出版信息

Am J Dermatopathol. 1986 Aug;8(4):314-20. doi: 10.1097/00000372-198608000-00007.

Abstract

Elastosis perforans serpiginosa (EPS) is now a well-recognized potential complication of long-term penicillamine therapy. By itself, EPS appears to be a relatively innocuous cutaneous side effect of penicillamine. However, suspicion has been raised in recent literature that EPS may represent only a superficial manifestation of more serious penicillamine-induced systemic elastic tissue damage, particularly involving blood vessels. This is a report of a patient with Wilson's disease who was treated with penicillamine for 14 years. She developed EPS, and histologic examination of the skin revealed the characteristic penicillamine-induced "lumpy-bumpy" elastic fibers in the dermis. More important, nonlesional skin showed the same elastic fiber changes. Of greatest significance was the finding of identical elastic fiber alterations in an artery.

摘要

匐行性穿通性弹力纤维病(EPS)现在是长期青霉胺治疗公认的潜在并发症。就其本身而言,EPS似乎是青霉胺相对无害的皮肤副作用。然而,最近的文献引发了怀疑,即EPS可能仅仅是更严重的青霉胺诱导的全身弹性组织损伤的一种表面表现,尤其是涉及血管。本文报告了一名患有威尔逊病的患者,她接受青霉胺治疗14年。她出现了EPS,皮肤组织学检查显示真皮中有特征性的青霉胺诱导的“凹凸不平”的弹性纤维。更重要的是,非病变皮肤显示出相同的弹性纤维变化。最具意义的是在一条动脉中发现了相同的弹性纤维改变。

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