Linear scleroderma as a rare cause of enophthalmos: a case report

INTRODUCTION: Enophthalmos is an important physical sign which can be easily missed. CASE PRESENTATION: A 64-year old female presented with painless and progressive shrinking of her right eye. Visual acuity was 6/6 in both eyes. The main clinical findings included exophthalmometry readings of 14 mm in the right eye and 22 mm in the left eye and a linear scar on her right forehead. This scar is a feature of linear scleroderma and called "en coup de sabre". She was referred to a dermatologist for further assessment. CONCLUSION: Enophthalmos is defined as the relative recession of the globe into the bony orbit and if measuring greater than 2 mm can give a noticeable cosmetic deformity. Scleroderma is a systemic or localised disease. Linear scleroderma has the following features-localised fibrosis of the skin, blood vessels, subcutaneous fat, muscle and sometimes bone. Histology shows an inflammatory and a sclerotic phase. Ophthalmic effects include enophthalmos, lash loss, lid induration or tightening and periorbital oedema.