Corneal manifestations of scleromyxedema

Bilateral superficial corneal deposits and scleromyxedema, an uncommon dermatosis caused by the accumulation of acid mucopolysaccharide in the skin often associated with a benign monoclonal gammopathy developed in a 71-year-old man. A biopsy specimen of the cornea showed that the deposits stained strongly positive for IgG and lambda chains. Ultrastructurally, the deposits consisted of amorphous granular material. Scleromyxedema should be considered in the differential diagnosis of noninflammatory superficial keratopathies associated with benign gammopathies.