Linear Morphea En Coup de Sabre

Linear scleroderma en coup de sabre (LSCS) is one of the subtypes of llinear scleroderma. It affects the frontal scalp. The term “en coup de sabre” was originally put forth to depict the types of injuries inflicted on soldiers who were struck on the head with a sword. The results of such injury was a thickened scar on one side of the forehead. LCSC often beings with a hypopigmented or hyperpigmented streak on the forehead. That streak may become red and then indurated over time.

LSCS typically affects in children (67%), with a slight predominance in girls (about 2:1). It usually first appears in the first two decades of life. Patients with LSCS ca sometimes have underlying neurologic abnormalities and seizures. Children with LSCS are often advised to undergo complete neurologic examination along with brain MRI as part of their evaluation.

Ulc et al, 2022

Authors from Poland set out to review all the data published in the medical literature pertaining to treatments used for LSCS.. The authors retrieved 34 articles that analyzed treatment outcomes. These included 4 retrospective cohort studies, 2 prospective cohort studies, 4 case series, and 24 case reports. These 34 articles represented a total of 69 patients (38 children and 31 adults).

Methotrexate was found to be the most commonly investigated treatment (26 patients) with a highest response rate (26/26, 100%). Other treatments included systemic glucocorticosteroids (nine patients), followed by UVA1 (four patients), mycophenolate mofetil (two patients), hydroxychloroquine (five patients), abatacept (two patients), tocilizumab (three patients), cyclosporine (one patient), interferon gamma (one patient), PUVA therapy (two patients), NB-UVB therapy (one patient), and pulsed dye laser (one patient).

In addition to the various medical treatments, reconstructive and surgery treatment was successfully used for lesions with settled disease activity to improve the cosmetic aspect of the lesions.


The overall conclusion of this study was that methotrexate is currently the most often-studied treatment for LSCS and is associated with good clinical outcomes.


Ulc E et al. Therapeutic and Reconstructive Management Options in Scleroderma (Morphea) en Coup de Sabre in Children and Adults. A Systematic Literature Review. J Clin Med. 2021 Sep 29;10(19):4517.

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