Localized scleroderma causing enophthalmos: A rare entity
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The following case has been reported in the Indian Journal of Ophthalmology.

A 27-year-old lady presented with painless progressive shrinking of left eye since 20 years, following orbital trauma with iron rod. She also had a left inguinal skin lesion 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.

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” 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.

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. 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.

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