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Kawakibi et al. Plast Aesthet Res 2023;10:48 https://dx.doi.org/10.20517/2347-9264.2022.120 Page 5 of 9

Body
The cutaneous manifestations of systemic sclerosis can involve the extremities and trunk. Transplantation
of ASCs in the upper and lower extremities can correct contour irregularities, improve dyschromia and
[66]
sensitivity, and stop disease progression .

LOCALIZED SCLERODERMA
Linear scleroderma manifests as an indurated linear deformity involving the face or extremities. Linear
scleroderma is a subtype of localized scleroderma, which presents as a depressed linear lesion due to
hardening of the skin, progressive loss of subcutaneous fat, and possible involvement of the muscle and
underlying bone . It typically affects children and young adults and occurs on the scalp, face, or
[67]
extremities. Scleroderma “En Coup de Sabre” is a subtype of linear scleroderma limited to the hemiface,
affecting the frontoparietal scalp and forehead . Other subtypes of limited scleroderma affect differing
[67]
depths of tissue in various areas of the body and include plaque morphea, generalized morphea, and deep
morphea . Traditionally, linear scleroderma has been treated with scar release, negative-pressure wound
[2]
therapy, and hyaluronic acid fillers . Skin excision with tissue expansion and primary closure yields
[67]
reasonable aesthetic results in patients with scleroderma “En Coup de Sabre”. However, it requires several
procedures and multiple clinic visits for tissue expansion, which can be burdensome to the patient . In
[68]
addition, if a noticeable scar remains on the forehead following the serial excision, this may be a suboptimal
aesthetic outcome for the patient. Clinicians have also attempted to use less invasive approaches to correct
the deformities related to scleroderma, including the use of hyaluronic acid fillers to correct contour
irregularities in the scalp . However, high resorption rates, the need for repeated injections, and high costs
[69]
are significant disadvantages.

More recently, AFT has provided an advantage compared to current treatment options for local
scleroderma as it improves structural volume and contour irregularities and has ASCs that improve the
quality of the overlying skin . AFT can present significant advantages over other surgical interventions,
[66]
including low donor site morbidity, uncomplicated postoperative course, and the ability to achieve natural
contours and durable results. Clinically, the treatment of linear scleroderma of the frontal scalp with AFT
demonstrates significant improvement in the appearance of the lesion with minimal reportion rates [70,71] . In
cases where forehead depressions associated with linear scleroderma are significant, serial AFT can be
considered or AFT in conjunction with implant-based reconstruction (porous polyethylene) can be used to
further improve contour irregularities . Our preferred method of treatment is serial AFT for linear
[69]
scleroderma, as this surgical procedure has proven to be effective with low donor site morbidity and low
complication rates.

SPECIAL CONSIDERATIONS FOR FAT GRAFTING IN SCLERODERMA
AFT is a safe and effective surgical technique for the treatment of cutaneous scleroderma. However, several
questions remain. Currently, there is no standardized protocol for fat graft harvesting, processing, or
injection methods. Variations between studies limit outcome comparisons and study generalizability.
Further research is required to establish AFT protocols in the scleroderma population to produce maximal
aesthetic and functional outcomes. This is currently limited by unclear fat retention rates. Current research
efforts involve investigating the optimal AFT techniques to identify factors that minimize graft resorption.

It has been well established that vascularity of the recipient site is an important factor for graft survival. The
involvement of blood vessels and fibrosis of the tissue in scleroderma can decrease vascularity in those
[72]
lesions and potentially negatively impact graft survival . Therefore, repeat AFT procedures can be
beneficial, where the cumulative pro-angiogenic benefit provided by fat grafts gradually improves recipient