Management Options for Linear Scleroderma (“En Coup de Sabre”)

Skorochod, Ron; Nesher, Gideon; Gronovich, Yoav

doi:10.1097/dss.0000000000003539

Cited by 4 publications

(4 citation statements)

References 34 publications

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“…Generally, there is a varying degree of atrophy of the deep subcutaneous fat as well as the bones at the lesions of ECDS. 10 , 16 However, for the first time to our knowledge, abnormal bony hyperplasia beneath the atrophic subcutaneous fat atrophy was found in a patient with ECDS. Unfortunately, despite the detection of the rare bony hyperplasia, we were not able to find the exact cause.…”

Section: Discussionmentioning

confidence: 90%

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Bony Hyperplasia Beneath Atrophic Soft Tissue: A Rare Case of En Coup de Sabre and Literature Review

Ma,

Huang,

Chen

et al. 2023

CCID

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In this study, we report a rare case of en coup de sabre with hyperplasia of the left frontal bone beneath skin lesion, which is detected during magnetic resonance imaging screening and preoperative evaluation. A 27-year-old woman with 13-year history of progressive soft tissue depression in the forehead and scalp, and was treated by traditional Chinese herb before the disease went into stationary stage. The patient underwent serial long-pulsed laser treatments and autologous fat grafting with satisfactory outcome. To our knowledge, this is the first time that bony hyperplasia beneath the soft tissue lesion was found in a patient with en coup de sabre.

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Section: Discussionmentioning

confidence: 90%

“…Common surgical treatments for ECDS include autologous fat grafting, local flaps, expanded scapular flap, etc. 16 Since the overall tissue deficit in the frontal region can be adequately compensated for with soft tissue reconstructions, it is not necessary to intervene on bone atrophy or hyperplasia from an aesthetic perspective.…”

Section: Discussionmentioning

confidence: 99%

Bony Hyperplasia Beneath Atrophic Soft Tissue: A Rare Case of En Coup de Sabre and Literature Review

Ma,

Huang,

Chen

et al. 2023

CCID

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“…Due to its effect on muscle tone, injections of botulinum toxin to the perioral and facial areas have been suggested as a treatment option to improve facial appearance. Yet, this therapy was not found to have an effect on facial tissue atrophy [38]. Cumsky et al [39] recently published their experience using a combination of hyaluronic acid filler and botulinum toxin.…”

Section: Discussionmentioning

confidence: 99%

Treatment of Scleroderma-Related Microstomia Using Hyaluronic Acid: An Interventional Study

Shir-az,

Berl,

Mann

et al. 2023

Life

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Systemic sclerosis (SSc) or scleroderma is a rare, systemic, autoimmune connective tissue disease. It causes increased collagen synthesis, leading to multi-organ sclerosis, including the skin and joints. Patients’ overall health and quality of life are harmed dramatically. Involvement of the face and, especially, the oral opening can limit patients’ ability to speak and eat, oral hygiene, and cosmetic appearance. Profhilo® (NAHYCO®) is an over-the-counter product consisting of pure hyaluronic acid. It is used to improve skin quality by increasing collagen production and adipocyte vitality. This interventional study evaluated the results of perioral injections of hyaluronic acid in terms of improved skin quality, elasticity, and increased oral opening. Patients diagnosed with SSc received an injection of one syringe of Profhilo® (2 mL of hyaluronic acid) at each of two clinic visits at one-month intervals. The oral opening was measured between the upper and lower central incisors before and after treatment. Quality of life was assessed using the modified Rodnan Skin Score and Health Assessment Questionnaire-Disability Index. A total of 14 patients received the first treatment, and 11 received the second treatment. The mean oral opening increased from 31.6 mm (range 17–50 mm) prior to therapy to 35.8 mm (range 21–56) 2 months following the second injection. Statistical analysis showed that there was a significant increase in the oral opening as observed one week (36.2 mm, p = 0.011), one month (36.2 mm, p = 0.007), and three months (31.6 mm, p = 0.023) after the second injection, at the 5-month follow-up. Treatment of SSc patients’ perioral area with Profhilo® can result in significant improvements in oral opening and quality of life.

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“…Morphological disorders may be accompanied by neurological and ophthalmological disorders as well as impaired functioning of the stomatognathic system. An increase in the degree of facial asymmetry and its changed proportions may lead to stigmatization, affecting the patient's psychosocial functioning [10].…”

Section: Introductionmentioning

confidence: 99%

Disturbances of the stomatognathic system and possibilities of its correction in patients with craniofacial morphea

Wróblewska,

Polańska,

Mojs

et al. 2023

pdia

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Morphea en coup de sabre and progressive hemifacial atrophy are extremely rare connective tissue disorders causing facial deformity. In extreme cases, morphological disorders are accompanied by symptoms of a clear impairment of the stomatognathic system. The aetiology of the above-mentioned diseases is still unknown. Properly planned therapy in the field of maxillofacial orthopaedics makes it possible to correct the asymmetric pattern of hard tissue growth and thus enable rehabilitation. The task of augmentation techniques is the volumetric supplementation of tissue defects resulting from atrophic processes. The degree of destruction and the extent of changes determine the method of correction. Mild and moderate defects are treated mainly with biomaterials and autologous adipose tissue. The severe course of hemifacial atrophy and morphea en coup de sabre and the associated significant tissue atrophy necessitate the search for more complex methods of treatment. In this paper, we summarize the disturbances of the stomatognathic system in patients with craniofacial morphea, together with an analysis of current treatment options.

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Management Options for Linear Scleroderma (“En Coup de Sabre”)

Cited by 4 publications

References 34 publications

Bony Hyperplasia Beneath Atrophic Soft Tissue: A Rare Case of En Coup de Sabre and Literature Review

Bony Hyperplasia Beneath Atrophic Soft Tissue: A Rare Case of En Coup de Sabre and Literature Review

Treatment of Scleroderma-Related Microstomia Using Hyaluronic Acid: An Interventional Study

Disturbances of the stomatognathic system and possibilities of its correction in patients with craniofacial morphea

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