Ophthalmic findings in linear scleroderma manifesting as facial en coup de sabre. (November 2018)

• Record Type: Journal Article
• Authors: Fledelius, Hans; Danielsen, Patricia; Ullman, Susanne
• Is Part Of: Eye. Volume 32:Number 11(2018)
• Page Start: 1688
• Page End: 1696
• Publication Date: 2018-11
• Subjects: Ophthalmology -- Periodicals
• DOI: 10.1038/s41433-018-0137-9
• Languages: English
• ISSNs: 0950-222X

Abstract

Abstract Background To evaluate ophthalmic involvement in a long-term series of patients with en coup de sabre (ECS) close to the eye based on the hypothesis that this is not commonly affected, or simply under-reported. Methods An observational study of ophthalmological findings in patients from Copenhagen University Dermatology Clinics. A standard eye examination further included exophthalmometry, axial length and keratometry (IOLMaster), and horizontal eye muscle thickness (B-scan ultrasonography). Results Thirty-one consecutive patients were included from 2014 to 2015 (25 females, 6 males; median age, 33 years; range, 11–71 years). Twenty-seven patients had undergone ophthalmic evaluation more than once (observation time, 1–31 years; median, 7 years). Most eyes were normal or had currently adapted to eventual adnexal lesions and to insidious changes in eye position and/or motility. However, significant ipsilateral complications had developed related to 8 eyes, where two patients had more than one disorder. The ophthalmic pathologies were: blind eye (n = 2) due to adult age keratopathy/perforation and to Coats-like retinal detachment in childhood; restricted eye motility and diplopia (n = 2); acquired corneal astigmatism (n = 2); and dense cataract with light sense only (n = 1). Two patients had optic neuritis-like presentations, and lacrimal sac pathology occurred in one. Conclusions The main ophthalmic focus possibly explained the high proportion of significant lesions in this patient series (in 8 of 31). In addition to the established feature of enophthalmos, the oculometric evidence suggested smaller eye and rectus muscle involvement, interpreted as a secondary (late) negative trophic effect of the overlying skin disorder on eye structures.