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Linear scleroderma en coup de sabre in an adult female

Ocular, adnexal and tomographic features

Shah, Sushmita G1,; Raichura, Nirav D1,2; Parikh, Komal3; Sonawane, Deepa4; Shah, Gaurav Y1

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doi: 10.4103/ijo.IJO_525_20
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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].

Figure 1
Figure 1:
(a) Photograph of the face, (b) Right side of scalp showing alopecia
Figure 2
Figure 2:
(a) Right enophthalmos [Hertel's exophthalmometry - (base 100) 12/16 mm] (b) Deep superior sulcus with mild retraction of upper lid with right exotropia (c) Restriction of adduction

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].

Figure 3
Figure 3:
(a) Slit lamp photograph showing localized thickening of the inferior corneal epithelium. (b) AS OCT showing epithelium with bullae, stromal edema and localized hyperreflectivity along the endothelium (c) Brownish pigmentation on endothelium along with ground glassy precipitates

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].

Figure 4
Figure 4:
CT scan of the orbits revealed right eye enophthalmos, focal osseous defect of lamina papyracea (red arrow), deficient orbital fat (white arrow), fibrosis of soft tissue (red star) along medial wall of right orbit


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.[12] 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.[3] Lid abnormalities due to fibrotic involvement of eye lids[1] and extraocular muscles[3] 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.

2.1 Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

2.2 Financial support and sponsorship


2.3 Conflicts of interest

There are no conflicts of interest.


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Suttorp Schulten MSA, Koornneef L. Lineal scleroderma associated with ptosis and motility disorders Br J Ophthalmol. 1990;94:694–5
Zannin ME, Martini G, Athreya BH, Russo R, Higgins G, Vittadello F, et al Ocular involvement in children with localised scleroderma: A multi-centre study Br J Ophthalmol. 2007;91:1311–4

Linear morphea; Linear scleroderma en coup de sabre

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