Abstract
Aim: To present a case of linear scleroderma (LS) involving the extraocular muscles and periorbita with follow-up over an 18-year period. Method: Initial presentation was of a female aged 9 years with progressive left unilateral ptosis which ultimately underwent surgical correction. Results: The main features of ‘en coup de sabre’ subsequently manifested on the left side of the face and head. An unusual feature was the characteristic subcutaneous cleft which also appeared on the opposite side above the right eyebrow. The left eye went on to develop a corneal ulcer with resulting poor visual acuity. An ipsilateral oculomotility disorder was then noted in addition to poor eyelid closure. Conclusion: This case highlights the need to exclude ‘en coup de sabre’ as a potential differential diagnosis in any case of progressive or recurrent ptosis, especially in childhood. As oculomotility may also be affected ultimately, it could lead to corneal compromise following ptosis correction.
Highlights
Linear scleroderma (LS) is an uncommon autoimmune disease which presents clinically as a ‘localised’ form in which purely local areas of skin are affected or a ‘systemic’ form where internal organs in addition to the skin are affected
Scleroderma ‘en coup de sabre’ is a term used to describe a lesion of LS which is localised to the frontal parietal section of the face and scalp
LS does not follow a set pattern of progression and can be associated with many different ocular abnormalities
Summary
Linear scleroderma (LS) is an uncommon autoimmune disease which presents clinically as a ‘localised’ form in which purely local areas of skin are affected or a ‘systemic’ form where internal organs in addition to the skin are affected. Lesions usually start with contraction and firmness of the skin over the affected area. An ivory irregular sclerotic plaque can subsequently develop, sometimes with telangiectatic vessels coursing over it together with hyperpigmentation at the edge.[1] Scleroderma ‘en coup de sabre’ is a term used to describe a lesion of LS which is localised to the frontal parietal section of the face and scalp. A linear depressed groove appears on the frontoparietal region extending into the scalp producing a linear zone of alopecia, which
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