Lattice-like paraproteinemic keratopathy (PPK) of monoclonal gammopathy of undetermined significance (MGUS)

Description

A male patient aged 71 years presented with a history of progressive visual loss in the past 3 years. Ophthalmological evaluation showed a best-corrected visual acuity of 20/200 bilaterally and the presence of bilateral linear lattice-like corneal opacities similar to lattice dystrophy (LD) and dense nuclear cataracts at slit lamp examination (figure 1). There was no family history of ocular diseases. Serum protein electrophoresis included in the systemic investigation showed a band in the -globulin fraction (figure 2). Nephelometry detected an increased level of light chains—75.6 mg/dL (normal: 3.3–19.4 mg/dL) and a normal level of light chains—14.4 mg/dL (normal: 5.7–26.3 mg/dL) and an increased / ratio of 5.25 (normal: 0.26–1.65). Immunoelectrophoresis also confirmed the IgG- gammopathy. Myelogram showed 2% of plasma cells. No lytic bone lesions were present on plain X-ray. No mutations were found on transforming growth factor β-induced gene (TGFBI) nor on gelsolin gene...

http://ift.tt/2lFDtZY