Moorman C M, McDonald B
Department of Neuropathology, Radcliffe Infirmary NHS Trust, Oxford, UK.
Eye (Lond). 1997;11 ( Pt 5):603-6. doi: 10.1038/eye.1997.162.
Conjunctival amyloidosis is an uncommon condition which usually arises secondary to chronic infection or trauma to the eye. It is rare to find significant deposits of amyloid involving the conjunctiva alone in the absence of a known antecedent infective, traumatic or hereditofamilial disorder. We report three patients with primary localised conjunctival amyloidosis. Each patient presented with a painless swelling in the lower fornix; in two this gradually extended to involve the bulbar and palpebral conjunctiva of both eyes with friable yellow deposits. Detailed clinical photographs illustrate the specific features of the disease. None of the patients had previous ocular pathology and bacterial, viral and fungal cultures of the conjunctiva were negative. There were no signs of inflammation. Conjunctival biopsies in each case showed large amorphous nodular deposits of amyloid within the substantia propria. Extensive systemic investigations including serum and urinary protein immunoelectrophoresis were normal. Follow-up ranged from 2 to 15 years after presentation. Treatment was symptomatic. As the typical features of amyloidosis present late in the course of conjunctival disease a diagnostic biopsy is often delayed. Once there is histopathological verification plasma cell disease must be excluded, although if the amyloid is limited to the conjunctiva systemic involvement is unlikely.
结膜淀粉样变性是一种罕见的疾病,通常继发于眼部的慢性感染或创伤。在没有已知的先前感染、创伤或遗传家族性疾病的情况下,仅发现结膜有大量淀粉样蛋白沉积是罕见的。我们报告了3例原发性局限性结膜淀粉样变性患者。每位患者均表现为下穹窿无痛性肿胀;其中2例肿胀逐渐蔓延至双眼的球结膜和睑结膜,伴有易碎的黄色沉积物。详细的临床照片展示了该疾病的具体特征。所有患者既往均无眼部病变,结膜的细菌、病毒和真菌培养均为阴性。无炎症迹象。每例结膜活检均显示固有层内有大量无定形结节状淀粉样蛋白沉积。包括血清和尿蛋白免疫电泳在内的广泛全身检查均正常。随访时间为就诊后2至15年。治疗以对症治疗为主。由于淀粉样变性的典型特征在结膜疾病病程后期才出现,诊断性活检往往会延迟。一旦有组织病理学证实,必须排除浆细胞疾病,尽管如果淀粉样蛋白仅限于结膜,发生全身累及的可能性不大。