Correa Leandro J, Maccio J Pablo, Esposito Evangelina, Monti Rodolfo, Gonzalez-Castellanos M Eugenia, Paradelo Martin, Serra Horacio M, Urrets-Zavalia Julio A
Department of Ophthalmology, University Clinic Reina Fabiola, Universidad Catolica de Cordoba, Oncativo 1248, Cordoba, 5000, Argentina.
Centro Medico de Patologia y Citopatologia, Cordoba, Argentina.
BMC Ophthalmol. 2015 Jul 19;15:77. doi: 10.1186/s12886-015-0075-2.
Conjunctival amyloidosis is a very rare condition, generally unilateral, and presents mostly as an isolated condition without systemic compromise. Our purpose is to present a new case of systemic amyloidosis with a bilateral conjunctival involvement.
A 66-years-old caucasian female complaining of conjunctival hemorrhage and chemosis in both eyes for the last five years had been discontinuously treated with topical antibiotics and corticosteroids without any evident improvement. She presented with a pink-yellow infiltration in the inferior conjunctiva of both eyes. Conjunctival biopsy under optical microscopy revealed amyloid deposit, confirmed by Congo red staining. Mucosal biopsy from esophagus and rectus confirmed amyloidosis by Congo red stain. Immunohistochemistry of bone marrow biopsy showed an increased number of plasma cells and an over-expression of light chain kappa subunit. She was treated with corticosteroids and lubrication with an improvement of symptoms. Ocular lesions remained stable after a follow-up of 3 years.
Conjunctival amyloidosis is a rare entity that may be overlooked, and should be differentiated from chronic conjunctivitis and conjunctival malignancies. Although it presents most frequently as a local process, a systemic involvement should always be ruled out.
结膜淀粉样变性是一种非常罕见的病症,通常为单侧性,大多表现为孤立性病症,无全身损害。我们的目的是报告一例双侧结膜受累的系统性淀粉样变性新病例。
一名66岁的白种女性,在过去五年中一直抱怨双眼结膜出血和结膜水肿,间断接受局部抗生素和皮质类固醇治疗,但无明显改善。她双眼下结膜出现粉黄色浸润。光学显微镜下结膜活检显示淀粉样沉积,刚果红染色证实。食管和直肠黏膜活检经刚果红染色证实为淀粉样变性。骨髓活检免疫组化显示浆细胞数量增加,轻链κ亚基过度表达。她接受了皮质类固醇治疗和润滑处理,症状有所改善。随访3年后眼部病变保持稳定。
结膜淀粉样变性是一种可能被忽视的罕见病症,应与慢性结膜炎和结膜恶性肿瘤相鉴别。虽然它最常表现为局部病变,但始终应排除全身受累情况。