PDF(Pubmed)
Abstract：
BACKGROUND: Paraproteinemic keratopathy is a rare disorder characterized by the bilateral accumulation of polychromatic deposits diffusely in all corneal layers together or not with diffuse or patchy pseudo lipid deposits. We present an atypical case of paraproteinemic keratopathy which lead to an initial misdiagnosis of infectious crystalline keratopathy.
METHODS: a 69-year-old woman with an asymptomatic keratopathy detected during a cataract intervention. Slit-lamp examination revealed several hyper refringent subepithelial foci with fern-shaped branches, resembling crystalline keratopathy, in her left eye. Anterior segment optical coherence tomography revealed exclusively subepithelial hyperreflective lesions limited to the anterior stroma. The progressive bilateralization and progression of the condition prompted us to include other entities with crystalline corneal deposits in our differential diagnosis. Hematological analysis showed a high number of free Kappa light chains. Despite the typical clinical appearance of crystalline keratopathy, the atypical evolution and test results led us to consider that monoclonal gammopathy could be the cause of this entity.
CONCLUSIONS: Paraproteinemic keratopathy may present in its early stages as a unilateral subepithelial crystalline keratopathy. Thus, it must always be taken into account in the differential diagnosis of any crystalline keratopathy, particularly when there are no predisposing factors for an infectious crystalline keratopathy. Early recognition of this rare entity is important to address the associated potentially serious systemic disease.
METHODS: a 69-year-old woman with an asymptomatic keratopathy detected during a cataract intervention. Slit-lamp examination revealed several hyper refringent subepithelial foci with fern-shaped branches, resembling crystalline keratopathy, in her left eye. Anterior segment optical coherence tomography revealed exclusively subepithelial hyperreflective lesions limited to the anterior stroma. The progressive bilateralization and progression of the condition prompted us to include other entities with crystalline corneal deposits in our differential diagnosis. Hematological analysis showed a high number of free Kappa light chains. Despite the typical clinical appearance of crystalline keratopathy, the atypical evolution and test results led us to consider that monoclonal gammopathy could be the cause of this entity.
CONCLUSIONS: Paraproteinemic keratopathy may present in its early stages as a unilateral subepithelial crystalline keratopathy. Thus, it must always be taken into account in the differential diagnosis of any crystalline keratopathy, particularly when there are no predisposing factors for an infectious crystalline keratopathy. Early recognition of this rare entity is important to address the associated potentially serious systemic disease.
摘要：
背景：副蛋白血症性角膜病变是一种罕见的疾病，其特征是在所有角膜层中弥漫性或不伴有弥漫性或斑片状假脂质沉积的双侧聚积。我们介绍了非典型的副蛋白性角膜病变病例，该病例导致感染性晶体性角膜病变的初步误诊。
方法：一名69岁女性在白内障介入治疗期间发现无症状角膜病变。裂隙灯检查显示有几个高折射率的上皮下病灶，带有蕨类植物状分支，类似于晶体性角膜病，在她的左眼。前段光学相干断层扫描显示仅限于前基质的上皮下高反射病变。病情的进行性双侧化和进展促使我们在鉴别诊断中包括其他具有晶体角膜沉积物的实体。血液学分析显示大量的游离Kappa轻链。尽管有典型的晶体性角膜病变的临床表现，非典型的进化和测试结果使我们认为单克隆丙种球蛋白病可能是该实体的原因。
结论：副蛋白血症性角膜病可能在其早期阶段表现为单侧上皮下晶体性角膜病。因此，在任何晶体性角膜病变的鉴别诊断中必须始终考虑到这一点，特别是当没有感染性晶体性角膜病变的诱发因素时。对这种罕见实体的早期识别对于解决相关的潜在严重全身性疾病很重要。
方法：一名69岁女性在白内障介入治疗期间发现无症状角膜病变。裂隙灯检查显示有几个高折射率的上皮下病灶，带有蕨类植物状分支，类似于晶体性角膜病，在她的左眼。前段光学相干断层扫描显示仅限于前基质的上皮下高反射病变。病情的进行性双侧化和进展促使我们在鉴别诊断中包括其他具有晶体角膜沉积物的实体。血液学分析显示大量的游离Kappa轻链。尽管有典型的晶体性角膜病变的临床表现，非典型的进化和测试结果使我们认为单克隆丙种球蛋白病可能是该实体的原因。
结论：副蛋白血症性角膜病可能在其早期阶段表现为单侧上皮下晶体性角膜病。因此，在任何晶体性角膜病变的鉴别诊断中必须始终考虑到这一点，特别是当没有感染性晶体性角膜病变的诱发因素时。对这种罕见实体的早期识别对于解决相关的潜在严重全身性疾病很重要。
