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


1. Katz KA. Frontal linear scleroderma (en coup de sabre). Dermatol Online J 2003;9:10
2. Copcu E. Treatment of coup de sabre deformity with porous polyethylene implant. Plast Reconstr Surg 2004;113:758Y759
3. Pekiner FN, Yu¨celten D, Gu¨mru¨ B, et al. Frontal linear scleroderma (en coup de sabre): a case report. J Dent Child (Chic) 2006;73:
4. Pace C, Ward SE, Pace A. A rare case of frontal linear scleroderma (en coup de sabre) with intra-oral and dental involvement. Br Dent J 2010;208:249Y250
5. Neuber GA. Fetttransplantation. Dtsch Ges Chir Kongr Verh 1893;22:66 6. Coleman SR. Facial recontouring with lipostructure. Clin Plast Surg 1997;24:347
6. Coleman SR. Facial recontouring with lipostructure. Clin Plast Surg 1997;24:347
7. Coleman SR. Hand rejuvenation with structural fat grafting. Plast Reconstr Surg 2002;110:1734Y1736
8. Coleman SR, ed. Structural Fat Grafting. St Louis, MO: Quality Medical Publishing, Inc, 2004
9. Moseley A, Zhu M, Hedrik MH. Adipose derived stem and progenitor cells as fillers in plastic and reconstructive surgery. Plast Reconstr Surg 2006;118:121Y128
10. Coleman SR. Structural fat grafting: more than a permanent filler. Plast Reconstr Surg 2006;118:110
11. Cortese A, Savastano G, Felicetta L. Free fat transplantation for facial tissue augmentation. J Oral Maxillofac Surg 2000;58:164
12. Coleman SR, Saboeiro A, Sengelmann R. A comparison of lipoatrophy and aging: volume deficits in the face. Aesthetic Plast Surg 2009;33:14Y21
13. Lantoni JD, Marshall DM, Wolfe AS. Overgrowth of fat autotransplantedf for correction of localized steroid-induced atrophy. Plast Reconstr Surg 2000;106:1566Y1569
14. Trepsat TF. Volumetric face lifting. Plast Reconstr Surg 2001;108:1358Y1370
15. Aust L, Devlin B, Foster SJ, et al. Yield of human adipose derived adult stem cells from liposuction aspirates. Cytotherapy 2004;6:7
16. Rigotti G, Marchi A, Sbarbati A. Adipose-derived mesenchymal stem cells: past, present, and future. Aesthetic Plast Surg 2009;33:271Y273
17. Fraser JK, Wulur I, Alfonso Z, et al. Fat tissue: an underappreciated source of stem cells for biotechnology. Trends Biotechnol 2006;24:150Y154
18. Clauser L, Tieghi R, Polito J, et al. Facial lipostructure in complex reconstructive surgery. Riv Ital Chir Plast 2005;37:75Y79
19. Clauser L. Optimizing maxillofacial and craniofacial results. In: Coleman SR, Mazzola RF, eds. Fat Injection From Filling to Regeneration. St Louis, MO: Quality Medical Publishing, Inc. 2009:475Y500
20. Clauser L, Tieghi R, Consorti G. Parry-Romberg syndrome: volumetric regeneration by structural fat grafting technique. J Craniomaxillofac Surg 2010;38:605Y609
21. Consorti G, Tieghi R, Franco F, et al. Endocrine orbitopathy (Basedow-Graves disease): bone deformity after decompression according to Matton. Tissue regeneration with lipostructure. Riv Ital Chir Plast 2009;7:91Y95
22. Clauser LC, Tieghi R, Galie` M, et al. Structural fat grafting: facial volumetric restoration in complex reconstructive surgery. J Craniofac Surg 2011;22:1695Y1701