Department of Ophthalmology, Basurto University Hospital, Avda. Montevideo s/n, Bilbao, E- 48013, Spain.
Department of Haematology, Basurto University Hospital, Bilbao, Spain.
BMC Ophthalmol. 2024 Jun 19;24(1):263. doi: 10.1186/s12886-024-03487-6.
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.
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.
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.
副蛋白血症性角膜病变是一种罕见的疾病，其特征是双侧角膜各层弥漫性积聚多色沉淀物，或伴有弥漫性或斑片状假性脂质沉积。我们报告了一例副蛋白血症性角膜病变的不典型病例，该病例最初误诊为感染性结晶状角膜病变。
一名 69 岁女性，在白内障干预期间发现无症状性角膜病变。裂隙灯检查显示左眼有数个上皮下高折射性病灶，呈蕨状分支，类似于结晶状角膜病变。眼前节光学相干断层扫描显示仅局限于前基质的上皮下高反射性病变。病情的进行性双侧化和进展促使我们将其他具有结晶状角膜沉积物的实体纳入鉴别诊断。血液分析显示游离 Kappa 轻链数量高。尽管典型的结晶状角膜病变的临床表现，但不典型的演变和检查结果促使我们认为单克隆丙种球蛋白血症可能是该疾病的原因。
副蛋白血症性角膜病变在早期可能表现为单侧上皮下结晶状角膜病变。因此，在任何结晶状角膜病变的鉴别诊断中都必须考虑到这一点，特别是在没有感染性结晶状角膜病变的诱发因素时。早期识别这种罕见的实体对于处理相关的潜在严重系统性疾病非常重要。
