Treatment of “En Coupe De Sabre “ Linear Scleroderma. Soft Tissue Augmentation and Regeneration by Autologous Fat Transfer

Luigi Clauser,MD1, Antonio Lucchi,MD1, Carolina Sannino,MD1, and Andrea Edoardo Bianchi,MD1, 2

1Unit of Maxillo-Facial Surgery, Istituto Stomatologico Italiano, Via Pace, 21, 20122 Milano, Italy;
2 University UniCamillus ,Via di Sant’Alessandro,8,00131 Roma,Italy

Aim: Frontal linear scleroderma, also known as ‘‘en coup de sabre,’’ is a congenital deformity characterized by atrophy and furrowing of the skin of the front parietal area above the level of the eyebrows. In most cases it occurs as a single paramedian line that may be associated with hypoplasia of underlying structures and facial hemiatrophy. In case of a wide lesion many reconstructive strategies have been proposed. The modern approach is aimed at augmentation of the tissue deficiency by using lipostructure and tissue regeneration. Autologous  fat transfer (AFT) seems to be an effective strategy to restore the normal volume and contour of the face while providing a source of adipose-derived stem cells (ADSCs) with a multilineage differentiation potential.

Methods: In this report, we present a rare case of linear scleroderma en coupe de sabre which was successfully managed with three stages of  autologous fat grafting.

Conclusion: The proposed uses for ADSCs in tissue repair and regeneration are quite impressive. Recent works on ADSCs would suggest that adult cells may prove to be an equally powerful regenerative tool in treating congenital and acquired maxillofacial disorders. More importantly, physicians, researchers and international associations need to cooperate in informing clinicians about what practices are based on evidence  and to encourage support of additional research. There is increasing interest in a possible therapeutic effect of ADSCs from processed lipoaspirate for a wide spectrum of clinical applications in the facial and craniofacial area. AFT can be used in any facial area where soft tissue is lacking or where there is scarring, producing natural and long-lasting results. Mesenchymal stem cells represent a great tool in regenerative medicine. However, more definitive studies are needed  to answer specific questions regarding the best technique to be used and the role of ADSCs. Autologous fat grafting provides a safe and easy approach for the treatment of linear scleroderma en coup de sabre, long-term clinically satisfactory results can be obtained.

Keywords: Autologous fat transfer, frontal linear scleroderma, congenital craniofacial malformations, reconstructive surgery, rare autoimmune diseases, tissue regeneration, linear scleroderma, en coup de sabre

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Luigi Clauser,Antonio Lucchi,Carolina Sannino,Andrea Edoardo Bianchi. Treatment of “En Coupe De Sabre“ Linear Scleroderma. Soft Tissue Augmentation and Regeneration by Autologous Fat Transfer.American Journal of Surgical Research and Reviews, 2021, 4:30. DOI:10.28933/ajsrr-2021-07-0103


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