Therapeutic and Reconstructive Management Options in Scleroderma (Morphea) en Coup de Sabre in Children and Adults. A Systematic Literature Review

Abstract

Scleroderma (morphea) en coup de sabre is a localized subtype restricted to the frontoparietal region of the head. Current treatment paradigms rely on low levels of evidence, primarily case reports and case series-supported by expert opinions. The aim of this article was to systematically analyze current data related to the treatment of localized scleroderma en coup de sabre. The databases Scopus, PubMed, and EBSCO were searched for all reports discussing the treatment of localized scleroderma en coup de sabre. The keywords en coup de sabre, “facial linear scleroderma”, and “morphea linearis”, combined with “treatment” or “therapy” were used as search terms. A total of 34 articles analyzed treatment outcomes for patients with localized scleroderma en coup de sabre including 4 retrospective cohort studies, 2 prospective cohort studies, 4 case series, and 24 case reports, representing a total of 69 patients (38 children and 31 adults). Methotrexate was 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). Reconstructive and surgery treatment was successfully used for lesions with settled disease activity to improve the cosmetic aspect of the lesions. Conclusion: methotrexate is the most often-studied treatment and reported good clinical outcomes in children and adults with localized scleroderma en coup de sabre.

Management Options for Linear Scleroderma (“En Coup de Sabre”)

“…The process eventually results in a hypopigmented furrow, accompanied by surrounding scarring alopecia. 3…”

“…Pharmacological agents, such as methotrexate, oral and topical corticosteroids, cyclosporine, mycophenolate mofetil, hydroxychloroquine, abatacept, and tocilizumab, have all been used to treat this condition, with varying results. 3…”

“…The process eventually results in a hypopigmented furrow, accompanied by surrounding scarring alopecia. 3 "En coup de sabre" may present with neurologic comorbid involvement in addition to the cutaneous findings, ranging from migraine to seizures and focal neurologic deficits. The neurological manifestations can precede the appearance of cutaneous lesions.…”

“…Pharmacological agents, such as methotrexate, oral and topical corticosteroids, cyclosporine, mycophenolate mofetil, hydroxychloroquine, abatacept, and tocilizumab, have all been used to treat this condition, with varying results. 3 Phototherapy in the form of psoralen and ultraviolet-A irradiation has also been proposed as a potential treatment agent and demonstrated positive results in trials and clinical settings. 5 Despite the diverse nature of treatment modalities, the addition of surgical and minimally invasive management is frequently required for the correction of residual cutaneous and osteogenic deformities.…”

A linear atrophic and hyperpigmented plaque on the face

“…Treatment options for burnt out/inactive lesions include autologous fat transfer and filler injection with hyaluronic acid or polymethylmethacrylate. [1] www.cosmoderma.org…”