Linear morphea of the forehead (en coup de sabre)

Linear morphea of the forehead or en coup de sabre (ECDS) is an unusual variant of morphea. It typically occurs in children although cases of adult-onset ECDS exist as reported here. ECDS has a specific distribution on the frontal scalp and forehead and is usually unilateral. Sclerosis in ECDS lesions may invade deeply to involve underlying muscle and bone and may exist on the same clinicopathologic spectrum as Parry-Romberg syndrome. Extracutaneous involvement is frequent, with neurologic and ophthalmologic findings occurring most commonly. The etiology of ECDS is unclear but may be autoimmune in origin. The question of whether Borrelia infection also plays a role remains controversial. Current evidence supports first-line treatment with methotrexate and oral glucocorticoids followed by phototherapy and mycophenolate mofetil.