Linear scleroderma with orbital involvement: follow up and magnetic resonance imaging.

Linear scleroderma ‘en coup de sabre’ with ptosis and oculomotility disorders: case report and review of the literature

“…Examining this case and the literature, both the onset of bilateral LS 7 and ocular muscle involvement seems to start in the 10-to 20-year age range. 11,13,15 Females appear to develop LS at least 3-8 times more frequently than males. 17 Areas involved in LS 'en coup de sabre' do not usually cross the midline of the body.…”

“…Several published articles have looked at the ocular impact of this disease [2][3][4][5][6][7][8][9][10][11][12][13][14][15][16] but no reviewed papers have documented the natural progression of LS and 'en coup de sabre' for as long as in this case.…”

“…4,7,9,11,[13][14][15][16] These have been due to either direct muscle infiltration or peripheral nerve disease. In scleroderma, increased collagen deposition occurs, resulting in dermal thickening.…”

“…9,13 One patient has been treated successfully with steroids and penicillamine; however, due to the very short follow-up it is not clear whether this improvement was permanent. 9 In another case of ineffective treatment cortisone therapy and radiotherapy was initiated but the patient complained of increasing pain around the right eye and became 'blind' on the right side.…”

“…'En coup de sabre' is an uncommon and seldom reported dermatological disorder; it is usually unilateral, but rare bilateral cases have been recorded. 7 It is often reported with ptosis or lid abnormalities [4][5][6][7][8][9][11][12][13][14][15][16] but concurrent oculomotor disorders are rare. 4,7,9,11,[13][14][15][16] Case report…”