Coats-like response in linear en coup de sabre scleroderma.

Purpose: To describe a Coats-like response and central nervous system vasculitis and its successful management in a patient presenting with en coup de sabre scleroderma.

Methods: Retrospective interventional case report. Chart review.

Results: A 20-year-old white male patient with en coup de sabre scleroderma on treatment with oral cyclophosphamide and prednisone for associated central nervous system inflammation presented with photopsias in the ipsilateral side of the scleroderma. A magnetic resonance imaging before presentation had shown lesions suggestive of localized central nervous system vasculitis. Ocular examination showed vitritis and multiple venous and arteriolar saccular dilatations with lipid exudation and subretinal fluid. These were treated with intravitreal bevacizumab, focal laser, and subtenon triamcinolone acetonide. After a year of follow-up, there was near resolution of the exudation and complete resolution of the vitreous inflammation, and the patient has been clinically stable with no visual loss.

Conclusion: Coats-like response with ocular inflammation can be associated with central nervous system vasculitis in patients with linear scleroderma. Careful clinical evaluation and aggressive multipronged treatment are necessary to ensure a successful outcome.