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原发性双侧结膜淀粉样变性患者的睑外翻和结膜肿物

Ectropion and Conjunctival Mass in a Patient with Primary Bilateral Conjunctival Amyloidosis.

作者信息

Meduri Alessandro, Rechichi Miguel, Mazzotta Cosimo, Scalinci Sergio Zaccharia, Jaroudi Mahmoud O

机构信息

Department of Ophthalmology, University of Messina, Messina, Italy.

Department of Ophthalmology, University of Magna Graecia, Catanzaro, Italy.

出版信息

Case Rep Ophthalmol Med. 2016;2016:5610753. doi: 10.1155/2016/5610753. Epub 2016 Nov 16.

Abstract

Amyloidosis is a group of disorders characterized by deposition of an extracellular protein, known as amyloid, in an abnormal fibrillar form with highly characteristic histopathologic staining properties. The clinical presentation can vary from a focal, localized lesion where amyloidosis has minor clinical consequences to extensive systemic disease that can involve any organ system of the body. Ocular amyloidosis can occur as a localized lesion or as a part of a systemic disorder. Conjunctival amyloidosis is an uncommon condition that is rarely associated with systemic disease. It may be a manifestation of an immunologic disorder. We report the case of a patient with bilateral conjunctival amyloidosis who was referred to us with the suspicion of a malignant conjunctival lesion. Examination of both eyes showed a yellow-pink mass with prominent intrinsic vessels, subconjunctival hemorrhage, and ectropion of the left lower eyelid. Diagnosis of primary localized conjunctival amyloidosis was made based on histopathologic evaluation of incisional biopsy and negative systemic work-up. Ocular amyloidosis is a rare disease that is slowly progressive and has a wide variety of clinical presentations. Consequently, the clinical diagnosis is often overlooked or delayed. Definitive diagnosis is achieved through histopathologic evaluation of biopsy specimen.

摘要

淀粉样变性是一组以细胞外蛋白(称为淀粉样蛋白)沉积为特征的疾病,其以异常的纤维状形式存在,具有高度特征性的组织病理学染色特性。临床表现从淀粉样变性具有轻微临床后果的局灶性、局限性病变到可累及身体任何器官系统的广泛全身性疾病不等。眼部淀粉样变性可表现为局限性病变或作为全身性疾病的一部分出现。结膜淀粉样变性是一种罕见的病症,很少与全身性疾病相关。它可能是免疫紊乱的一种表现。我们报告了一例双侧结膜淀粉样变性患者,该患者因疑似结膜恶性病变转诊至我们处。双眼检查显示有一个黄粉色肿物,其内部血管突出,结膜下出血,左下眼睑外翻。根据切开活检的组织病理学评估和全身检查阴性结果,诊断为原发性局限性结膜淀粉样变性。眼部淀粉样变性是一种罕见疾病,进展缓慢,临床表现多样。因此,临床诊断常常被忽视或延迟。通过活检标本的组织病理学评估可实现明确诊断。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9571/5126439/e6e45262b131/CRIOPM2016-5610753.001.jpg

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