A case series of linear scleroderma en coup de sabre with eyelid involvement
Author: Li Teng Kok
Base Hospital / Institution: Moorfields Eye Hospital
ePoster presentation
Abstract ID: 25-183
Purpose
Linear scleroderma en coup de sabre (ECDS) is a rare subtype of localised scleroderma characterised by linear atrophy and sclerosis of the skin and underlying subcutaneous tissue affecting the frontoparetial region above the brow. ECDS lesions have rarely been reported to extend into the upper eyelid. We aim to describe three cases of ECDS with eyelid involvement, including the clinical presentations and surgical management to improve symmetry.
Methods
Clinical notes were reviewed retrospectively.
Results
Three cases of inactive ECDS with mean age of 28.7 years presented between 2017 and 2023. None had symptoms of systemic disease. All three patients presented with a linear depression in the forehead, running from the scalp into the eyelid, affecting the left forehead and eyelid in two patients and the right side in one. Patient 1 showed signs of left upper eyelid scarring and retraction, with sulcus hollowing and lid lag. Patient 2 presented with a left ptosis, sulcus hollowing and flattening of the supraorbital ridge. Patient 3 had right upper lid retraction, lash and brow loss and associated upper lid skin telangiectasia. Computed tomography scans demonstrated subcutaneous tissue loss in the brow and upper lid region, with thinning of the frontal cortical bone under the affected area. Patients 1 and 2 were treated with autologous fat transfer (AFT) to the affected forehead and brow area, with ipsilateral lid lowering and posterior ptosis repair respectively. Patient 3 had injectable filler to the right temple and sulcus and a contralateral ptosis repair. Eyelid tattooing also improved the appearance of lash loss on the affected upper eyelid. All three patients had significantly improved forehead and eyelid symmetry with no post-operative complications at mean follow up period of 28.6 months.
Conclusion
ECDS is rare and can extend into the upper eyelid. The role of oculoplastics in the inactive phase to restore symmetry is important. We describe the use of a combination of autologous fat transfer and injectable filler to the forehead and brow, and lid lowering or contralateral ptosis correction to restore eyelid symmetry.
Additional Authors
| First name | Last name | Base Hospital / Institution |
|---|---|---|
| Mohsan | Malik | Moorfields Eye Hospital |
| Tessa | Fayers | Western Eye Hospital |
| Konstantina | Sorkou | The Ezra Clinic |
| Alia | Issa | The Ezra Clinic |
| Daniel | Ezra | Moorfields Eye Hospital |
