Article Text

Download PDFPDF

Linear scleroderma with orbital involvement: follow up and magnetic resonance imaging
Free
  1. K RAMBOER,
  2. PH DEMAEREL,
  3. A L BAERT
  1. Department of Radiology
  2. University Hospitals, Leuven, Belgium
  3. Department of Ophthalmology
  4. University Hospitals, Leuven, Belgium
  1. I CASTEELS,
  2. G DRALANDS
  1. Department of Radiology
  2. University Hospitals, Leuven, Belgium
  3. Department of Ophthalmology
  4. University Hospitals, Leuven, Belgium
  1. Ph Demaerel, MD, Department of Radiology, University Hospitals, Herestraat 49, B-3000 Leuven, Belgium.

Statistics from Altmetric.com

Editor,—Scleroderma is a chronic connective tissue disease of unknown aetiology.1 Linear scleroderma is an uncommon dermatological disorder which produces an unilateral and localised band-like linear atrophy of the skin and its underlying structures.

Orbital involvement has been described, but is very rare.12

CASE REPORT

A 21-year-old woman first presented to an ophthalmologist because of a progressive narrowing of the right palpebral fissure over a period of 3 years with loss of lashes of the eyelids. Further ophthalmic examination was normal. An orbital computed tomogram (CT) was normal.

Three years later there was additional enophthalmia and an entropion of the lower right eyelid was seen (Fig 1). Visual acuity was 8/10 in the right eye and 10/10 in the left. Visual field examination and colour vision were normal.

Figure 1

Right eye: note the narrowing of the palpebral fissure and the loss of lashes of the eyelids.

With the exception of a localised area of parietotemporal alopecia on the right side, no other skin defects could be detected. General examination revealed no muscular pain, no arthralgia, no fatigue, and no other systemic symptoms. Laboratory examination of muscle enzymes, tissue antibodies and complement factors was normal. There were no inflammatory factors and thyroid tests were within normal limits.

On an orbital CT the right eye was displaced laterally by a band-like mass. The medial rectus muscle and oblique superior muscle could not be identified. Muscle and conjunctival biopsy only showed some oedema in these structures.

The presumed diagnosis of localised scleroderma was confirmed by a dermatological examination. Initially no treatment …

View Full Text

Request Permissions

If you wish to reuse any or all of this article please use the link below which will take you to the Copyright Clearance Center’s RightsLink service. You will be able to get a quick price and instant permission to reuse the content in many different ways.