Asymptomatic Anterior Uveitis without Systemic Activity in en Coup de Sabre Scleroderma: A Case Report

Linear scleroderma en coup de sabre is a rare subtype of localized scleroderma that may present with extracutaneous manifestations, including neurological and ophthalmological involvement. While uveitis has been described, it typically occurs in conjunction with active systemic or cutaneous disease. Asymptomatic anterior uveitis in the setting of long-standing remission is a rare occurrence. This report presents a pediatric case of linear scleroderma characterized by asymptomatic anterior uveitis that was asynchronous with systemic disease activity.

We report the case of a 9-year-old boy with a history of linear scleroderma who was successfully treated with methotrexate. One year after discontinuing immunosuppressive therapy and while in complete clinical systemic remission, the patient presented with asymptomatic, unilateral non-granulomatous anterior uveitis, detected incidentally during a routine ophthalmologic examination. Extensive serologic and infectious workup excluded alternative etiologies. The reintroduction of methotrexate and topical corticosteroids resulted in the complete resolution of inflammation.

This case highlights the potential for delayed and asymptomatic uveitis in pediatric patients with linear scleroderma en coup de sabre, even after systemic remission. Given that up to 30% of patients may develop ocular involvement, regular follow-up with ophthalmology should be ensured, regardless of systemic disease activity. Early detection is crucial for preventing visual impairment and should be incorporated into the long-term, multidisciplinary follow-up of these patients.