Management Options for Linear Scleroderma ("En Coup de Sabre").

Abstract

En coup de sabre (ECDS) is a form of linear-scleroderma, primarily affecting the face. Despite effectiveness of pharmacological interventions in the management of lesions, sequelae of cutaneous deformities are common. Review the existing surgical tools for the correction of "en coup de sabre" ECDS lesions and facial deformities. The authors conducted a literature search for reports on surgical interventions for ECDS deformations, in the electronic databases of PubMed, Scopus, and Cochrane Library Databases. Twenty-six publications reporting 39 patients were found in the literature. The average age of patients was 25 ± 4.7 years. Thirty-one of the patients were females. Six methods were found in the literature for surgical correction of ECDS. Fat grafting was performed in 41% of patients, surgical reconstruction in 18%, injectable fillers in 15%, implants in 10%, botulinum toxin injections in 8%, and bone/cartilage grafts in 8%. Imaging studies of the skull and face were performed in 36% of patients. Bony defects were found in 64.3% of them. Different surgical and minimally invasive options exist in the armamentarium of clinicians correcting ECDS deformities. Tailoring the method of correction to patient's needs and expectation is crucial in ensuring patient satisfaction.