A case of adult onset linear scleroderma, en coup sabre variant responsive to methotrexate monon-treatment: A review of treatment options

Linear scleroderma “en coup de sabre”(ECDS) is a rare variant form of morphoea with an annual incident of about 1 per 100,000. The onset of disease is usually in the adolescent population and three times more likely to occur in females than males. ECDS can present as solitary or bilateral linear lesions along the paramedian forehead or frontoparietal scalp sometimes involving the hairline. The disease is characterised by an active inflammatory phase followed by a progressive sclerotic phase resulting in disfiguring scarring of the skin involved. In the more aggressive forms deeper tissue including fat, muscle, bone and neural tissue with subsequent seizures and or ophthalmological defects have been reported1.
While the exact aetiology of the disease is poorly understood, treatment options exist, however these are derived from small case reports, series retrospective or prospective studies. To date the following medications reportedly used in linear scleroderma include, topical steroids, topical tacrolimus, psoralen cream, calcipotriol oral glucocorticoids in combination with methotrexate, mycophenolate and ultraviolet radiation with equivocal results2. However, there currently are no randomised double blind controlled trials to assess the best treatment options, especially for the ECDS variant form of morphoea. Here we report a case and review of the current treatments for linear scleroderma ECDS variant.
We report a 27-year-old female with a rare adult onset ECDS variant of linear scleroderma successfully treated with methotrexate mono therapy for twelve months. Overall there was improvement in the inflammatory and sclerotic phase of the disease.
1. 1. Fette N, Werth V. Update on morphea. Part I. Epidemiology, clinical presentation and pathogenesis. J Am Acad Dermatol. 2011;64:217-28.
2. 2. Fette N, Werth V. Update on morphea. Part II. Outcome measures and treatment. J Am Acad Dermatol. 2011;64:231-42.

Dr. Jesse Johnston