Orbital Involvement in Linear Scleroderma: A Review of The Literature With an Unusual Case

Linear scleroderma "en coup de sabre" (ECDS) is an uncommon form of localized craniofacial scleroderma, with orbital involvement being exceptionally rare. We present a 29-year-old female with right-sided ECDS who presented with progressive vision loss. Examination revealed an enlarged blind spot with global depression, 6 mm of enophthalmos, ptosis, circumferential optic disc edema, an Adie's pupil, and a characteristic cutaneous ECDS lesion. MRI revealed severe orbital fat atrophy, enlargement of extraocular muscles, and optic nerve contortion with postcontrast enhancement, with no bony abnormalities or inflammation. The patient was managed conservatively with corticosteroids. A literature review identified 26 cases of orbital ECDS, most commonly presenting with enophthalmos, extraocular muscle atrophy, and occasionally bone remodeling. This case highlights several uncommon manifestations of ECDS in the orbit, particularly paradoxical enlargement of the extraocular muscles. These findings expand the known clinical spectrum of orbital disease in ECDS and highlight the need for regular ophthalmic monitoring.