A 42-year-old female presented to us with complaints of irritation, nocturnal lagophthalmos and a white spot in her right eye of 1-month duration. She was a known case of linear morphea diagnosed by scalp biopsy, elsewhere. We diagnosed her as a case of linear scleroderma en coup de sabre (LSCS). We describe her ocular, adnexal and tomographic features.
A 42-year-old female presented with complaints of irritation, nocturnal lagophthalmos and a white spot in her right eye. She had undergone cataract surgery with intraocular lens implantation in the right eye elsewhere, 8 months prior. She had been diagnosed with linear morphea from scalp biopsy 13 years prior.
Her best-corrected visual acuity was 6/9, N6 and 6/6, N6 in the right and left eye, respectively. Linear band-like skin indentation on the right frontal region with underlying bony defect, madarosis of medial half of her right eyebrow and eye lashes of the upper lid [Fig. 1a] and alopecia on same side of scalp was noted [Fig. 1b]. She also had right enophthalmos, deep superior sulcus, mild retraction of upper lid with right extropia and restriction of adduction in her right eye [Fig. 2a-c].
Slit-lamp examination revealed localized corneal epithelial thickening, bullae with stromal edema [Fig. 3a] which was also noted on anterior segment optical coherence tomography (AS OCT) [Fig. 3b]. There was a focal area of brownish pigmentation on the endothelium underlying the area of stromal edema with an adjoining area of round glassy precipitates [Fig. 3c].
A diagnosis of linear scleroderma en coup de sabre (LSCS) with focal corneal edema was made. She was advised topical 1% prednisolone acetate eye drop in a tapering dosage along with night time patching. Follow-up visit after 2 weeks showed a reduction in epithelial bullae. Computed tomography (CT) scan orbits revealed right enophthalmos with focal osseous defect in the floor of the right frontal sinus and lamina papyracea, deficiency of orbital fat and fibrosis of soft tissue along the medial wall of right orbit [Fig. 4].
LSCS is a rare disease with onset in the first two decades of life characterized by band-like linear atrophy of skin and underlying bone involving frontoparietal region. LSCS is a subtype of localized scleroderma or morphea and is autoimmune in origin. Common ocular manifestations include anterior uveitis of autoimmune origin and episcleritis due to inflammatory subconjunctival fibrosis. Lid abnormalities due to fibrotic involvement of eye lids and extraocular muscles may occur. Enophthalmos, madarosis, localized skin thinning, focal corneal edema and bony defects in orbital wall were the most conspicuous ocular, adnexal and tomographic findings in our patient.
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