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

机译:原发性双眼结膜淀粉样变性患者的外翻和结膜肿块

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摘要

Background. 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. Case Report. 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. Conclusion. 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.
机译:背景。淀粉样变性病是一组疾病,其特征在于以异常纤维状形式的胞外蛋白沉积,称为淀粉样蛋白,具有高度特征性的组织病理学染色特性。临床表现可能有所不同,从淀粉样变性病影响较小的局灶性局部病变到可能累及人体任何器官系统的广泛系统性疾病。眼淀粉样变性病可发生为局部病变或系统性疾病的一部分。结膜淀粉样变性是一种罕见疾病,很少与全身性疾病相关。它可能是免疫疾病的表现。案例报告。我们报告了双侧结膜淀粉样变性病患者的病例,该患者因怀疑患有恶性结膜病变而被转介给我们。两只眼睛的检查均显示为黄色粉红色块,内在血管明显,结膜下出血和左下眼睑外翻。基于切开活检的组织病理学评估和全身阴性检查,对原发性局部结膜淀粉样变性病进行诊断。结论。眼淀粉样变性病是一种罕见的疾病,缓慢进展,具有多种临床表现。因此,临床诊断常常被忽视或延迟。明确诊断是通过对活检标本进行组织病理学评估来实现的。

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