Localized scleroderma 'en coup de sabre' with external eye muscle involvement at the same line

Abstract

Summary A case of localized scleroderma ‘en coup de sabre’ in the right side of the front associated with myopathy of external eye muscles (levator palpebrae and rectus superior) at the same line is reported. It is discussed whether a predisposition to the disease might be laid down in the mesenchyme in early foetal life before the differentiation of the anatomical structures.

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.…”

“…David et al 9 proposed that possible causative factors can include trauma, infection or severe psychological stress. Serup et al 11 described a case involving the right side of the face associated with myopathy of the ipsilateral eye muscles (levator palpebrae and superior rectus). They hypothesised that a predisposition to the disease might be laid down in the mesenchyme in early foetal life before the differentiation of the anatomical structures.…”

“…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.…”

“…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.…”

“…'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…”