Frosted branch angiitis: a review.

The purpose of this study is to present the first report of a case of primary frosted branch angiitis from the UK and to review the characteristics of this rare disease. Primary frosted branch angiitis causes characteristic florid translucent retinal perivascular sheathing of both arterioles and venules in association with variable uveitis, retinal oedema and visual loss, normally with good recovery. A total of 57 cases have been reported in the world literature. Atypical, typically focal frosted branch angiitis may also occur secondary to other causes of intraocular inflammation, especially cytomegalovirus retinitis. Primary frosted branch angiitis has a characteristic presentation but a variable course, typically affecting children or young adults. The disease is likely to represent a common immune pathway in response to multiple infective agents. The optimal treatment is unclear.