关键词: Marshall syndrome acquired cutis laxa acquired elastolysis elastic fibers review

来  源:   DOI:10.1111/ijd.17338

Abstract:
Acquired cutis laxa (ACL) is a rare, nonhereditary cutaneous disorder characterized by saggy inelastic skin. It has been associated with various inflammatory, autoimmune, and neoplastic diseases, in addition to certain infections and medication. This article reviews ACL the demographical, clinical, and histological features of ACL, focusing on all associated disorders. Additionally, this review article provides an in-depth discussion of all the mechanisms implicated in the pathogenesis of ACL and all therapeutic options available; we also present an algorithm for the workup of patients with ACL. A systematic literature review was performed on PubMed/Medline and EMBASE databases, searching for all available articles on ACL with no limits on participant age, race, sex, nationality, or publication date. Ninety-eight articles were included. The total number of included patients was 110, with a mean age of 36.4 years at presentation (range 0.25-78) and a M:F sex ratio of 1.24. ACL was most commonly associated with inflammatory disorders (43%) followed by neoplastic disorders (27%). In 73% of the neoplastic-associated cases, ACL occurred on average 2.4 years before malignancy onset. ACL occurs months to years after an underlying inflammatory disorder. In 10% of the cases, ACL was associated with a particular drug, and in 2%, it was associated with specific infections. Data were derived from case reports, case series, letters to editors, observational studies, and abstracts. Limitations include the accuracy of published data, potential patient selection, and reporting bias. Dermatologists should be alert to these associations to provide adequate screening and management of patients with ACL.
摘要:
获得性皮肤松弛(ACL)是一种罕见的,以松弛无弹性皮肤为特征的非遗传性皮肤疾病。它与各种炎症有关,自身免疫,和肿瘤疾病,除了某些感染和药物治疗。本文回顾了ACL的人口统计学,临床,和ACL的组织学特征,专注于所有相关疾病。此外,这篇综述文章对ACL的发病机制和所有可用的治疗选择进行了深入讨论;我们还提出了ACL患者的检查算法.对PubMed/Medline和EMBASE数据库进行了系统的文献综述,搜索ACL上的所有可用文章,没有参与者年龄限制,种族,性别,国籍,或发布日期。其中包括98篇文章。纳入的患者总数为110,平均年龄为36.4岁(范围为0.25-78),M:F性别比为1.24。ACL最常见于炎症性疾病(43%),其次是肿瘤性疾病(27%)。在73%的肿瘤相关病例中,ACL平均发生在恶性肿瘤发病前2.4年。ACL发生在潜在的炎性疾病后数月至数年。在10%的案例中,ACL与特定药物有关,在2%中,它与特定感染有关。数据来自病例报告,案例系列,给编辑的信,观察性研究,和摘要。限制包括公布数据的准确性,潜在的患者选择,和报告偏见。皮肤科医生应警惕这些关联,以提供对ACL患者的充分筛查和管理。
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