SEARCH

SEARCH BY CITATION

Introduction

  1. Top of page
  2. Introduction
  3. Report of a case
  4. Discussion
  5. References

En coup de sabre is a localized variant of scleroderma that presents as a linear, atrophic depression affecting the frontoparietal aspect of the face and scalp. Occasionally, involvement of underlying structures, including muscle, bone, and rarely meninges and brain, occurs resulting in medical morbidities for the patient.[1] Given that this variant occurs on the face, however, it is often the cosmetic aspect for which patients seek out care and intervention. Indeed, the disfigurement and both its psychological and social effects may have a significantly negative impact on the patient's quality of life.[2]

Unfortunately, treatment for active en coup de sabre is difficult, and for stable lesions, attempts at improving cosmesis have led to suboptimal results.[3] Surgical excision, autologous fat grafting, autologous bone grafting, and placement of synthetic tissue inserts have been performed with varying degrees of success.[4-7] Dermal filler treatments offer an attractive option as they are much less invasive and possess distinct advantages compared to the aforementioned techniques. Hyaluronic acid filler is particularly well suited for soft tissue augmentation because of its tolerability, availability, relatively low cost, reversibility, and efficacy in volumization.[8] Upon review of the literature, we encountered two cases in which hyaluronic acid filler was utilized in the correction of hemifacial atrophy seen in Parry–Romberg syndrome, a distinct but related variant of linear scleroderma.[9, 10] However, these were both in combination with other modalities: in one case autologous fat transfer and in the other, calcium hydroxylapatite filler.[9, 10] Specifically in regard to en coup de sabre, there is a case report of hyaluronic acid filler used in conjunction with AlloDerm tissue matrix, which is essentially cadaveric dermis.[4] Herein, we report a novel case of en coup de sabre in which hyaluronic acid filler alone was successfully used to correct the atrophic defect.

Report of a case

  1. Top of page
  2. Introduction
  3. Report of a case
  4. Discussion
  5. References

A rheumatologist referred a 44-year-old caucasian female to our dermatology clinic for treatment considerations for the en coup de sabre variant of linear morphea. Onset of her clinically apparent disease began at age 34, and her lesion had been stable for several years by the time of her visit. She denied any previous cosmetic treatment of her condition. Upon physical exam, a linear vertical depression was present on her left medial forehead and scalp with a hypopigmented scar on the lower part. Associated alopecia and atrophy of the bony skull were also noted.

image

Figure 1. Right-sided view of patient at baseline, 14 d post filler injection and 7 months post-injection

Download figure to PowerPoint

image

Figure 2. Left-sided view of patient at baseline, 14 d post filler injection and 7 months post-injection

Download figure to PowerPoint

image

Figure 3. Straight on view of patient at baseline, 14 d post filler injection and 7 months post-injection

Download figure to PowerPoint

After a lengthy discussion of the risks, benefits, and alternatives, including the off-label use of hyaluronic acid filler, the patient was injected with two vials of hyaluronic acid intradermally to correct the depression on her left medial forehead (Figs. 13). The injections were well tolerated, and the patient did not experience any adverse effects. Immediate improvement in volume depletion was evident, and the patient expressed great satisfaction with the result. Three months following injection, the patient still retained significant cosmetic improvement and remained happy with the results. The patient received further injections six months after the initial treatment and again voiced her satisfaction. The patient's consent for publication of the case and her pictures was obtained.

Discussion

  1. Top of page
  2. Introduction
  3. Report of a case
  4. Discussion
  5. References

The en coup de sabre variant of linear morphea presents as an atrophic linear streak that is most often located on the paramedian forehead and scalp.[10] While single lesions are most common, multiple lesions may coexist in a single patient, with reports suggesting that the lesions may follow Blaschko's lines.[1, 11] The lesions commence as contractions and firmness of the skin over the affected area. This is followed by the development of an ivory-colored, irregularly shaped, sclerotic plaque, which often has hyperpigmentation at the periphery. Finally, profound atrophic changes may be noted leading to a permanent, depressed defect.[12-14]

The length of the active, inflammatory stage typically ranges from 2 to 5 years.[12, 13] Attempts at halting the progression during this phase have led to the use of a variety of pharmacologic therapies, including topical, intrale-sional, or systemic glucocorticoids, antimalarials, retinoids, penicillamine, penicillins, phenytoin, griseofulvin, calcitriol, interferon, and methotrexate. Other modalities such as phototherapy and physiotherapy have also been employed.[14-18] Although several regimens have shown benefit in case series, no controlled trials have been performed.

Cosmetic correction of stable en coup de sabre using several techniques has been variably effective in isolated case reports and small case series.[4-7, 9, 19] The use of hyaluronic acid filler as monotherapy in patients with en coup de sabre has been reported only once to our knowledge.[20] In addition, hyaluronic acid fillers have been used as an adjunct to concomitantly used implants or other types of filler in cases of linear scleroderma of the face as follows.

A case of Parry–Romberg syndrome, a variant of localized scleroderma characterized by facial hemiatrophy treated with calcium hydroxylapatite filler, has been reported by Cox and Soderberg.[9] Their approach was modeled after a recent prospective study of 30 patients by Caruthers and Caruthers that outlined the benefits of calcium hydroxylapatite injections for HIV-associated facial lipoatrophy.[19] Cox and Soderberg report using five injections of calcium hydroxylapatite at approximately 4-week intervals. The patient also received a single injection of hyaluronic acid in an attempt to correct volume depletion. Significant improvement in volume depletion was noted and the patient was “very satisfied” with the result.[9] In a recent report by Lane et al., upper lip injections of hyaluronic acid filler were given to a patient with Parry–Romberg syndrome. This filler improved cosmetic appearance and was used before a planned autologous fat transfer.[10] Finally, the use of hyaluronic acid filler as an adjunctive agent has been reported in a patient with en coup de sabre who received a synthetic tissue implant with AlloDerm. In this case, Robitschek et al. used hyaluronic acid filler to smooth out the borders of the implant and create a more even forehead contour.[4]

In terms of the cosmetic correction of atrophic skin lesions, hyaluronic acid offers many advantages. Hyaluronic acid is the most prominent glycosaminoglycan in the skin. When injected into the skin, it volumizes, softens, and hydrates the skin by potently binding to water. In addition to these benefits, it plays a role in cell growth, membrane receptor function, and adhesion.[8] It has also been shown to stimulate collagen production, which may explain why some patients, in our experience, with injectable hyaluronan appear to have some permanent improvement after multiple treatments.[21] While typically used for the improvement of age-related changes, it may also be used off label on a compassionate basis for the benefit of patients such as ours who suffer from atrophy as a sequela of a disease process. It must be noted, however, that in some areas of en coup de sabre lesions, the skin may be somewhat tethered to underlying structures. For these particularly bound down areas, filler alone will not suffice to correct the defect, and more invasive procedures may be required. In fact, at both the inferior and superior aspects of our patient's lesion, this was the case, and therefore the improvement is primarily noted in the central portion. The cosmetic improvement is nonethe-less substantial. Ongoing injections will be required to maintain the cosmetic outcome, which may indeed improve with repeated treatment.

In summary, we feel that hyaluronic acid filler may be safely and successfully used as monotherapy for temporary cosmetic improvement of en coup de sabre lesions. The benefit is most prominent in well-selected patients who may experience atrophy but in whom the prominent feature is not tethering to underlying structures.

References

  1. Top of page
  2. Introduction
  3. Report of a case
  4. Discussion
  5. References