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D-青霉胺诱导的匐行穿通性弹性纤维瘤 18 年随访。

An 18-year follow-up of a case of D-penicillamine-induced Elastosis perforans serpiginosa.

出版信息

Int J Immunopathol Pharmacol. 2011 Jan-Mar;24(1):257-9. doi: 10.1177/039463201102400133.

DOI:10.1177/039463201102400133
PMID:21496412
Abstract

Elastosis perforans serpiginosa (EPS) is a rare complication of chronic therapy with a high-dose of D-penicillamine (1 g daily for more than 5 years), characterized by the elimination of abnormal elastic fibers from the upper dermis through the epidermis. D-penicillamine (DPA) is a heavy metal chelator primarily used for disorders such as cystinuria and Wilson disease. This therapy can lead to induction of EPS through a still unknown mechanism. We report the follow-up of a D-penicillamine-induced EPS in patient with Wilson disease, which prompted us to switch the therapy with trientine (another metal chelator). After 14 years the cutaneous lesions are still visible; therefore, we conclude that the DPA-induced cutaneous damage is irreversible.

摘要

穿孔性弹性组织离解症(EPS)是一种罕见的并发症,与大剂量 D-青霉胺(每天 1 克,超过 5 年)慢性治疗有关,其特征是通过表皮从真皮上层消除异常弹性纤维。D-青霉胺(DPA)是一种重金属螯合剂,主要用于胱氨酸尿和威尔逊病等疾病。这种治疗方法可能通过一种尚未明确的机制导致 EPS 的发生。我们报告了一例由 D-青霉胺引起的威尔逊病患者的 EPS 随访情况,这促使我们改用三乙烯四胺(另一种金属螯合剂)进行治疗。14 年后,皮肤病变仍然可见;因此,我们得出结论,DPA 引起的皮肤损伤是不可逆转的。

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