Linear Scleroderma of the Head - Updates in management of Parry Romberg Syndrome and En coup de sabre: A rapid scoping review across subspecialties

Glaser, Daniel; Schutt, Christina; VonVille, Helena; Schollaert-Fitch, Kaila; Torok, Kathryn S.

doi:10.5152/eurjrheum.2019.19183

Cited by 13 publications

(12 citation statements)

References 69 publications

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“…In the latest analysis of the literature assessing the current procedure regarding the correction of en coup de sabre and hemiatrophia faciei Parry-Romberg defects, surgical procedures were the most frequent (59%). Autologous fat grafting was most commonly used as a cosmetic procedure (50% of procedures), followed by free flap transfers (24%) [45]. Because the method we applied can be a fast and comparatively painless means for the improvement of the patient's appearance, its popularisation should be given due consideration.…”

Section: Discussionmentioning

confidence: 99%

The Correction of Facial Morphea Lesions by Hyaluronic Acid: A Case Series and Literature Review

Owczarczyk‐Saczonek¹,

Kasprowicz-Furmańczyk²,

Kruszewska³

et al. 2020

Dermatol Ther (Heidelb)

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Introduction: The aim of the study is to assess the long-term safety and efficacy of hyaluronic acid (HA) administration in correction of facial morphea lesions and to review the literature on the subject. Morphea is a chronic inflammatory disease of the connective tissue which may lead to serious deformations. The lesions located on the face particularly affect patients' quality of life and self-esteem; thus, there is a demand for safe and effective methods of treatment. Case Presentation: The paper presents three female patients aged 16, 17 and 70 with facial morphea lesions who had HA preparation Juvéderm Ò Voluma or Volux, Vycross Ò technology, Allergan, injected. One of the patients had additionally fractional ablative CO 2 laser (FAL) therapy. Discussion: The literature provides reports on successful use of HA, polymethylmethacrylate and poly-L-lactic acid for the correction of facial defects in localized scleroderma. HA is a natural component of the extracellular matrix and it therefore minimizes the probability of immunogenicity. The application technique also plays an important role. On the other hand, FAL therapy leads to the degradation of the abnormal collagen and the induction of normal collagen synthesis. Conclusions: HA injection and combination of HA application with FAL are minimally invasive, effective and safe therapeutic options for patients suffering from morphea.

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Section: Discussionmentioning

confidence: 99%

The Correction of Facial Morphea Lesions by Hyaluronic Acid: A Case Series and Literature Review

Owczarczyk‐Saczonek¹,

Kasprowicz-Furmańczyk²,

Kruszewska³

et al. 2020

Dermatol Ther (Heidelb)

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“…It has great potential for application in morphea affecting the head and neck, where facial asymmetry can be difficult to evaluate clinically, and the LoSCAT often falls short in correctly quantifying disease activity and damage. Advanced analysis of images can be used in conjunction with clinical assessment to provide information about vascularity and pigmentation to further monitor disease activity (90). 3D imaging is portable, fast, easy to use, and inexpensive, and has many applications in facial morphea.…”

Section: Imaging Measures Of Morpheamentioning

confidence: 99%

Morphea: progress to date and the road ahead

et al. 2021

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Morphea is a rare autoimmune condition causing inflammation and sclerosis of the skin and underlying soft tissue. It is characterized by periods of activity (inflammation admixed with fibrosis), ultimately resulting in permanent damage (pigment change and tissue loss). Damage resulting from unchecked activity can lead to devastating, permanent cosmetic and functional sequelae including hair loss; cutaneous, soft tissue and bony atrophy; joint contractures; and growth restriction of the affected body site in children. This makes the early identification of activity and initiation of appropriate treatment crucial to limiting damage in morphea. To this end, recent investigative work has focused on validation of clinical, biomarker, imaging, and histologic outcomes aimed at accurately quantifying activity and damage.Despite promising results, further work is needed to better validate these measures before they can be used in the clinic and research settings. Although there has been recent approval of less toxic, targeted therapies for many inflammatory skin conditions, none have been systematically investigated in morphea.The mainstays of treatment for active morphea are corticosteroids and methotrexate. These are often limited by substantial toxicity. The paucity of new treatments for morphea is the result of a lack of studies examining its pathogenesis, with many reviews extrapolating from research in systemic sclerosis. Recent studies have demonstrated the role of dysregulated immune and fibrotic pathways in the pathogenesis of morphea, particularly interferon (IFN) gamma related pathways. Active morphea lesions have been foundto display an inflammatory morphea signature with CXCR3 receptor ligands, as well as a distinct fibrotic signature reflecting fibroblast activation and collagen production. CXCL9 and 10 have been associated with increased measures of disease activity. While immune dysfunction is thought to play the primary role in morphea pathogenesis, there are other factors that may also contribute, including genetic predisposition, environmental factors, and vascular dysregulation. There remains an essential need for further research to elucidate the pathogenesis of morphea and the mode of action of dysregulated upstream and downstream immune and fibrotic pathways. These studies will allow for the discovery of novel biomarkers and targets for therapeutic development.

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“…The role of autoimmune inflammation in the pathogenesis of these diseases is suggested by histology, cerebral fluid findings, and by the response to immunosuppressive agents. 1 The prescription of disease-modifying treatments is becoming more common, although the overall quality of available clinical studies for treatment of LS is low. 2 Different disease monitoring techniques (ultrasound, thermography, optical coherence tomography) have been proposed but have not been validated in large-scale studies, 1 and the use of magnetic resonance imaging (MRI) to assess disease activity in the jaw or periodontal region has not been reported.…”

Section: Introductionmentioning

confidence: 99%

“…1 The prescription of disease-modifying treatments is becoming more common, although the overall quality of available clinical studies for treatment of LS is low. 2 Different disease monitoring techniques (ultrasound, thermography, optical coherence tomography) have been proposed but have not been validated in large-scale studies, 1 and the use of magnetic resonance imaging (MRI) to assess disease activity in the jaw or periodontal region has not been reported. Here, we describe the case of a boy with refractory PRS/ECDS with significant involvement of the jaw and resorption of dental roots who responded to tocilizumab with significant reduction of bone edema as evidenced on MRI.…”

Section: Introductionmentioning

confidence: 99%

Parry Romberg disease with En Coup de Sabre Scleroderma: Effect of tocilizumab on periodontal bone inflammation

Meneghetti

Silva

Kluppel³

et al. 2020

Journal of Scleroderma and Related Disorders

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Parry Romberg disease and En Coupe de Sabre Scleroderma are frequently associated disorders that affect the face and can cause severe aesthetic and functional impairment. Systemic immunosuppression is the gold standard of first-line treatment in the pediatric rheumatology standpoint although it is often delayed in the pediatric dermatology clinics and more often used in cases of refractory neurological impairment. We report on a case with dental root resorption and severe periodontal bone inflammation detected on magnetic resonance imaging, which was successfully treated with the anti-IL-6 agent tocilizumab.

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Linear Scleroderma of the Head - Updates in management of Parry Romberg Syndrome and En coup de sabre: A rapid scoping review across subspecialties

Cited by 13 publications

References 69 publications

The Correction of Facial Morphea Lesions by Hyaluronic Acid: A Case Series and Literature Review

The Correction of Facial Morphea Lesions by Hyaluronic Acid: A Case Series and Literature Review

Morphea: progress to date and the road ahead

Parry Romberg disease with En Coup de Sabre Scleroderma: Effect of tocilizumab on periodontal bone inflammation

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