En coup de sabre — a case report of therapeutic difficulties

Abstract

En coup de sabre (ECDS) is a rare linear form of localized scleroderma that occurs more frequently in children than in adults. The involvement of the scalp leads to cicatricial alopecia. The aim of this paper is to present a problematic case of ECDS in young female patient. Many terapeutical methods were introduced, based on guidelines described in diagnostic and therapeutic recommendations of the Polish Dermatological Society (e.g. methotrexate, oral and intravenous glucocorticoids, subcutaneous triamcinolone injections and topical high potent steroids as well as kalcineurin inhibitor (tacrolimus). The lasting improvement was not achieved. The remission was finally induced after the implementation of mycophenolate mofetil.

En coup de sabre linear scleroderma – diagnostic difficulties. Case report

“…Frontal linear scleroderma (morphea en coup de sabre) is a rare disease from the group of limited connective tissue diseases, which is most prevalent in children and females (1)(2)(3) . The incidence is estimated at less than 3 per 100,000 (4) .…”

“…In the clinical picture, a unilateral, erythematous plaque is initially observed, which then transforms into a discoloured, deep furrow. Over time, it covers not only the skin, but also involves the subcutaneous tissue, muscles and bones (1,7,8) . The lesion initially begins at the level of the superciliary arch and reaches the fronto-parietal area, where the focus of scarring alopecia usually appears.…”

“…MRI abnormalities have also been reported, even in patients without concomitant neurological disorders (5,8,10) . Cases of maxillary and mandibular involvement with tooth root deformation and atrophy have also been described (1,2) . En coup de sabre may coexist in 20-40% of patients with progressive hemifacial atrophy (Parry-Romberg syndrome), which involves the skin, subcutaneous tissue, muscles and bones of half the face.…”

“…oral prednisone at 0.5-1.0 mg/kg body weight per day for 2-4 weeks or methylprednisolone in the form of intravenous pulses at 30 mg/kg per day, up to a maximum of 1,000 mg/day for 3 consecutive days per month for 3-6 months, are the treatment of choice. In the case of first-line treatment failure, the treatment may be supplemented with or switched to mycophenolate mofetil (MMF) at a dose of 1-2 g/day (1) . The probable mechanism of action of methotrexate in the treatment of scleroderma is to influence the expression of cytokines.…”