Coats-like response in linear en coup de sabre scleroderma

Area of Science:

• Ophthalmology
• Neurology
• Rheumatology

Background:

• En coup de sabre scleroderma is a rare subtype of localized scleroderma.
• Central nervous system (CNS) involvement in scleroderma, though uncommon, can be severe.
• Ocular manifestations in scleroderma are diverse and can impact vision significantly.

Purpose of the Study:

• To report a case of en coup de sabre scleroderma with concurrent Coats-like response and CNS vasculitis.
• To describe the successful management of these complex ocular and neurological conditions.

Main Methods:

• Retrospective case report with chart review.
• Patient presented with visual disturbances (photopsias).
• Diagnostic imaging (MRI) revealed CNS vasculitis; ocular examination showed vitritis and exudative changes.

Main Results:

• The patient, a 20-year-old male with en coup de sabre scleroderma, developed ocular symptoms.
• Ocular findings included vitritis, vascular dilatations, lipid exudation, and subretinal fluid, consistent with a Coats-like response.
• Treatment with intravitreal bevacizumab, laser, and subtenon triamcinolone resulted in resolution of inflammation and exudation over one year, preserving vision.

Conclusions:

• A Coats-like response and ocular inflammation can be associated with CNS vasculitis in patients with linear scleroderma.
• Aggressive, multi-modal treatment is crucial for managing these sight-threatening complications.
• Early diagnosis and intervention are key to achieving successful outcomes and preventing visual loss.