Demirci Hakan, Shields Carol L, Eagle Ralph C, Shields Jerry A
Oncology Service, Thomas Jefferson University, Philadephia, PA 19107, USA.
Surv Ophthalmol. 2006 Jul-Aug;51(4):419-33. doi: 10.1016/j.survophthal.2006.04.007.
Conjunctival amyloidosis is an uncommon condition that occasionally is associated with systemic involvement. The clinical presentations of conjunctival amyloidosis are diverse. We present six patients with conjunctival amyloidosis who were referred to us with the suspicion of another conjunctival lesion. The conjunctival lesion was circumscribed in two patients and diffuse in four patients. The lesion color was yellow in one patient and yellow-pink in five patients. The associated features were intrinsic vascularization (six patients), recurrent subconjunctival hemorrhage (four patients) and blepharoptosis that involved the palpebral conjunctiva (two patients). Systemic evaluation revealed primary systemic amyloidosis in one patient and no related systemic abnormalities in five patients. Management consisted of complete excisional biopsy for the two circumscribed lesions and incisional biopsy for the four diffuse lesions. Two patients with diffuse involvement showed progressive involvement of the conjunctiva over 3 years following incisional biopsy and the other four patients remained stable. Additionally, there was no systemic involvement in five patients. In conclusion, conjunctival amyloidosis generally manifests as a yellowish-pink, hemorrhagic mass deep to the epithelium. Most patients show no evidence of systemic amyloidosis.
结膜淀粉样变性是一种罕见的疾病,偶尔与全身受累有关。结膜淀粉样变性的临床表现多种多样。我们报告了6例结膜淀粉样变性患者,他们因疑似其他结膜病变而转诊至我们处。2例患者的结膜病变为局限性,4例为弥漫性。病变颜色在1例患者中为黄色,在5例患者中为黄粉色。相关特征包括固有血管化(6例患者)、复发性结膜下出血(4例患者)和累及睑结膜的上睑下垂(2例患者)。全身评估显示1例患者患有原发性系统性淀粉样变性,5例患者无相关全身异常。治疗包括对2例局限性病变进行完整切除活检,对4例弥漫性病变进行切开活检。2例弥漫性受累患者在切开活检后3年内结膜病变呈进行性发展,其他4例患者病情稳定。此外,5例患者无全身受累情况。总之,结膜淀粉样变性通常表现为上皮下黄粉色出血性肿块。大多数患者无系统性淀粉样变性的证据。