Possible IgG4-related disease in a case of necrotizing scleritis

Necrotizing scleritis can be a diagnostic and therapeutic challenge. We present a case of a 51-year-old female, who had necrotizing scleritis treated for 9 years with intermittent corticosteroid therapy. In view of its recalcitrant nature, she underwent scleral biopsy, which revealed histopathological features suggestive of IgG4- related disease. This case report highlights the importance of including IgG4-related disease as a differential diagnosis of scleritis and the role of scleral biopsy as a diagnostic tool in unresponsive cases.