Localized scleroderma 'en coup de sabre' and iridopalpebral atrophy at the same line

A case is reported of scleroderma 'en coup de sabre' in a 13-year-old girl with atrophy of the nasal part of the iris and loss of cilia on the upper eyelid. The lesions of the front and eye were located on precisely the same line. The line did not follow the innervation fields of the cranial, peripheral or autonomous nerves. Nor did it follow the tension lines of the skin (Langer), or underlying anatomical structures. It is discussed that the predisposition to the 'coup de sabre' line seen in localized scleroderma was laid down in the mesenchyme in early foetal life before the differentiation of the anatomical structures.