Corneal melt as the initial presentation of primary Sjogren's syndrome

Corneal melting is a rare complication of Sjogren's syndrome (SS). Previous ly reported cases of corneal ulceration occurred in patients with establish ed SS, usually secondary to RA. We describe the first case of corneal ulcer ation with stromal melting as the initial presentation of primary SS. A 79- year-old man without prior sicca symptoms developed a large sterile corneal ulcer that required extensive treatment over several months with ocular lu bricants, systemic immunosuppressives. and surgical repair. Evaluation For an underlying connective tissue disease revealed positive antinuclear antib odies (1:640 speckled) and anti-SSA antibody. A lip biopsy established the diagnosis of SS. Ulceration later occurred in the contralateral eye. Two ye ars after the last corneal ulcer and no longer taking prednisone, the patie nt's ocular disease remained quiescent taking azathioprine 175 mg and hydro xychloroquine 400 mg daily. This case highlights the potential for primary SS to present with serious ocular complications despite lack of a priori si cca symptoms, as well as the importance of immunosuppressive therapy in the treatment of this complication.