Scleroderma characterised by linear “coup de saber” is a variant of localized scleroderma that occurs in the hemiface, although it can be bilateral [1]. First described by Addison in 1854, it peaks in the fifth decade of life. Children are often diagnosed between the ages of 2 and 14 years, and females are more likely to be affected than males [2]. In addition to scarring on the forehead and scalp, the disease causes atrophy of the skin and subcutaneous tissues, including muscles, tendons, and bones [3]. Treatment relies primarily on immunosuppressants and corticosteroids [1,2].

This is a 38-year-old female patient, chronic smoker, who consulted for a hypopigmented lesion of the forehead, evolving for one year, progressively increasing in size, painless and not pruritic. Dermatologic examination revealed a well-limited, roughly rounded, hypochromic plaque with a smooth, slightly atrophic surface, 3 cm in size, located on the forehead (Fig. 1a). Dermatoscopy showed a slightly erythematous background with perifollicular pigmentation (Fig. 1b). Upon examination, a skin biopsy revealed plaque morphea. The patient was put on Calcipotriol + Betamethasone for 4 months. The patient was put on Calcipotriol + Betamethasone for 4 months. The plaque worsened and increased in size, becoming more depressed, with a sclerosed surface responsible for a non-scarring alopecic plaque with a positive traction sign at the periphery (Fig. 1c). In addition, the patient complained of headaches and visual fog for 4 months, so a craniofacial MRI showed no abnormalities. The patient was then put on a bolus of solumedrol and injectable methotrexate (15 mg/week) with positive progress.

Figure 1: (a) Well-limited hypochromic plaque, roughly rounded located on the right forehead. (b) Dermatoscopy showed a slightly erythematous background, with perifollicular pigmentation. (c) A depressed linear plaque, well-limited and sclerosed, located on the right side of the forehead.

Although coup de sabre morphea is a rare entity, it requires more research to identify the exact pathophysiology, as well as effective therapeutic options, for improving aesthetic outcomes and preventing long-term psychological damage.