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Year : 2018  |  Volume : 66  |  Issue : 11  |  Page : 1611-1612

Localized scleroderma causing enophthalmos: A rare entity

1 Ophthalmic Plastic Surgery and Ocular Oncology Services, LV Prasad Eye Institute, Vijayawada, Andhra Pradesh, India
2 Department of Dermatology, Venereology and Leprosy, Alluri Sitarama Raju Academy of Medical Sciences, Eluru, Andhra Pradesh, India

Date of Submission10-Apr-2018
Date of Acceptance16-Aug-2018
Date of Web Publication25-Oct-2018

Correspondence Address:
Dr. Anasua Ganguly Kapoor
Ophthalmic Plastic Surgery and Ocular Oncology Services, LV Prasad Eye Institute, Vijayawada - 521 137, Andhra Pradesh
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ijo.IJO_303_18

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Keywords: Enophthalmos, orbit, scleroderma

How to cite this article:
Kapoor AG, Kumar SV, Bhagyalakshmi N. Localized scleroderma causing enophthalmos: A rare entity. Indian J Ophthalmol 2018;66:1611-2

How to cite this URL:
Kapoor AG, Kumar SV, Bhagyalakshmi N. Localized scleroderma causing enophthalmos: A rare entity. Indian J Ophthalmol [serial online] 2018 [cited 2019 Jun 16];66:1611-2. Available from:

A 27-year-old lady presented with painless progressive shrinking of left eye since 20 years [Figure 1]a and [Figure 1]b, following orbital trauma with iron rod. She also had a left inguinal skin lesion [Figure 1]c since last 15 years. There was no history of any orbital surgery or any systemic symptoms. On ocular examination, her visual acuity was 20/20 both eyes. Slit lamp examination both eyes were normal except for few corneal glassy discrete endothelial precipitates [Figure 1]d. Hertel's exophthalmometry measured 15 mm and 10 mm in right and left eye, respectively. Periocular sensations were normal on both sides. She had a band like diagonal line on left forehead that is a form of localized scleroderma also known as “en coup de sabre” [Figure 1]a - black arrow] because of its resemblance with a sword wound. However, both sides of the face looked symmetrical. Fundus examination in left eye revealed retinal folds in macula [Figure 1]e. Axial length in both eyes was 21 mm. Computed tomography scan orbit showed gross enophthalmos in the left eye with reduced retrobulbar fat volume with no sign of any orbital fracture or any orbital mass [Figure 1]f and [Figure 1]g. On general examination, there was no palpable breast lump, but she had sclerotic skin lesions in left inguinal region that had been diagnosed as morphea type of localized scleroderma on skin biopsy by a dermatologist depending on presence of sclerosis of the skin and underlying tissues owing to excessive collagen deposition, adnexal atrophy, and mononuclear cell infiltrates. Systemic work up and autoantibody profile were negative. Intraorbital hyaluronic acid gel injection or autogenous fat transfer was suggested for orbital volume augmentation. However, she preferred observation and was not keen for any intervention. At 3 years follow-up, patient was status quo. Localized scleroderma, which manifested in both forms of linear scleroderma and morphea in this case, involved the orbit causing orbital fat atrophy leading to enophthalmos. Absence of hemifacial atrophy ruled out the diagnosis of  Parry-Romberg syndrome More Details in this case.
Figure 1: (a and b) Clinical photographs showing gross enophthalmos of left eye with linear diagonal scar over left forehead (Coup de Sabre-black arrow); (c) atrophic and sclerotic skin lesion in left inguinal region; (d) slit lamp photograph of left eye showing corneal endothelial precipitate; (e) fundus photograph of left eye showing multiple retinal folds at macula; (f and g) computed tomography scan orbit (axial and saggital section) showing enophthalmic left eye with reduced retrobulbar fat volume with absence of any other abnormality

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  Discussion Top

Scleroderma refers to a heterogeneous group of autoimmune fibrosing disorders. The disease can be either “localized” to the skin or “systemic” involving other organs in addition to the skin. Localized scleroderma can be further divided into linear and morphea (localized or generalized patches of atrophic and sclerotic skin lesions).[1] Linear scleroderma is characterized by localized fibrosis of skin, blood vessels, subcutaneous fat, muscle, and sometimes bone. It primarily affects the pediatric population in the first and second decade.[2] When linear scleroderma occurs on the fronto-parietal scalp, it is referred to as “'en coup de sabre,” given the resemblance of the skin lesion to the stroke of a sabre.[3] Specific neurological and ophthalmic associations of craniofacial scleroderma have been reported in literature that were all ruled out in our patient. The ophthalmic manifestations in scleroderma may include ptosis, lid induration or tightening, lash loss, motility disorder, keratoconjunctivitis sicca, conjunctival injection and vascular sludging, fornix shortening, iritis, heterochromia iridis, papillitis, retrobulbar pain, enophthalmos, displacement of outer canthus from resorption of orbital bone, and retinal hemorrhages.[2],[3],[4],[5]

  Conclusion Top

Scleroderma is a rare cause of enophthalmos after ruling out other likely differentials of orbital fracture and orbital metastatic breast cancer. The linear scar on the forehead “en coup de sabre” should not be discarded as an innocuous finding rather should aid the examiner in making the correct diagnosis.

Declaration of patient consent

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

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Conflicts of interest

There are no conflicts of interest.

  References Top

Kirkali PA, Kansu T, Sanac AS. Unilateral enophthalmos in systemic scleroderma. J Clin Neuroophthalmol 1991;11:43-4.  Back to cited text no. 1
Fernando BS, Cannon PS, Tumuluri K, Cook AE. Linear scleroderma as a rare cause of enophthalmos: A case report. J Med Case Rep 2007;1:179.  Back to cited text no. 2
Holland KE, Steffes B, Nocton JJ, Schwabe MJ, Jacobson RD, Drolet BA, et al. Linear scleroderma en coup de sabre with associated neurologic abnormalities. Pediatrics 2006;117:e132-6.  Back to cited text no. 3
West RH, Barnett AJ. Ocular involvement in scleroderma. Br J Ophthalmol 1979;63:845-7.  Back to cited text no. 4
Hock LE, Kontzialis M, Szewka AJ. Linear scleroderma en coup de sabre presenting with positional diplopia and enophthalmos. Neurology 2016;87:1741-2.  Back to cited text no. 5


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