Necrotizing scleritis is the least common but most dangerous and destructive form of scleritis. Necrotizing scleritis is an inflammatory condition affecting the sclera that is often associated with underlying systemic collagen vascular disease, vasculitis, or autoimmune disease. Rheumatoid arthritis is the most common condition associated with scleritis.

Necrotizing scleritis typically causes severe injection and pain in a patch of the sclera. With progressive disease, the sclera will necrose, leaving only a white patch of thin sclera surrounded by red, inflamed blood vessels. As the sclera thins, the blue color of the underlying uveal blood vessels becomes visible (blue sclera), and, at times, the intraocular pressure can cause an out-pouching of an area of the sclera.

Rarely, necrotizing scleritis can occur without pain and injection and may lead to ocular perforation. This form of necrotizing scleritis is called scleromalacia perforans.

Necrotizing scleritis is most commonly seen in middle-aged women with rheumatologic disease and is associated with worsening vasculitis. As with other forms of scleritis, there is a tendency for both eyes to be affected, although the disease may progress differently in each eye and at different rates.

Ocular or extraocular complications (including premature death) will be seen in over 50% of patients with necrotizing scleritis.