• Archives of Craniofacial Surgery
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• v.18 no.3
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• pp.166-171
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• 2017

Background: Localized scleroderma is characterized by a thickening of the skin from excessive collagen deposits. It is not a fatal disease, but quality of life can be adversely affected due to changes in skin appearance, joint contractures, and, rarely, serious deformities of the face and extremities. We present six cases of localized scleroderma in face from our surgical practice. Methods: We reviewed six localized scleroderma cases that were initially treated with medication and then received follow-up surgery between April 2003 and February 2015. Six patients had facial lesions. These cases presented with linear dermal sclerosis on the forehead, oval subcutaneous and dermal depression in the cheek. Results: En coup de sabre (n=4), and oval-shaped lesion of the face (n=2) were successfully treated. Surgical methods included resection with or without Z-plasty (n=3), fat graft (n=1), dermofat graft (n=1), and adipofascial free flap (n=1). Deformities of the affected parts were surgically corrected without reoccurrence. Conclusion: We retrospectively reviewed six cases of localized scleroderma that were successfully treated with surgery. And we propose an algorithm for selecting the best surgical approach for individual localized scleroderma cases. Although our cases were limited in number and long-term follow-up will be necessary, we suggest that surgical management should be considered as an option for treating scleroderma patients.

• Archives of Craniofacial Surgery
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• v.20 no.2
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• pp.112-115
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• 2019

Linear scleroderma en coup de sabre (LScs) is a variant of localized scleroderma. This disease typically occurs in patients in their 20s or younger individuals and predominantly occurs in the forehead area. A 26-year-old man with linear scleroderma was surgically treated at our center with Medpor (porous polyethylene) and dermal fat graft for the forehead lesion. After 26 months of postoperative follow-up, the depressed lesion that appeared scarred as well as the margins improved significantly. The surgical treatment of LScs using Medpor and dermal fat graft is an effective treatment modality that can increase patient satisfaction.

• Journal of the korean academy of Pediatric Dentistry
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• v.32 no.2
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• pp.256-261
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• 2005

Scleroderma is a connective tissue disease of unknown etiology, but known as a kind of auto-immune disease. It is most common in women especially in $30{\sim}50$ years, and very rare in childhood. It can be classified into two main classes : localized scleroderma & systemic sclerosis. Localized type has better prognosis, and usually involves skin only, or in some cases, the muscle below, except inner organs. Systemic type involves skin, oral mucosa and major internal organs. Involving facial skin, we can see small and sharp nose, expressionless stare and narrow oral aperture. Usually they have Raynaud's phenomenon, and in progress, show mouth opening limitation and sclerosis of tongue and gingiva. It is called CREST syndrome showing calcinosis cutis, Raynaud's phenomenon, esophageal dysfunction, sclerodactyly, and telangioectasia. Treatment of scleroderma is systemic and localized steroid therapy, use of collagen-link inhibitor (D-penicillamine), immune depressor and etc. Mouth opening limitation can be improved by mouth stretching exercise. We report a 6 years old boy, diagnosed with localized scleroderma who had mouth opening limitation. We could get additional mouth opening, and have done successful restorative treatment of mandibular and maxillary 1st and 2nd deciduous molar under deep sedation with nitrous oxide and enflurane.

• Maxillofacial Plastic and Reconstructive Surgery
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• v.37
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• pp.29.1-29.7
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• 2015

Facial asymmetry is found in patients with or without cosmetic facial alterations. Some patients have facial asymmetry that manifests underlying skeletal problems, while others have only limited soft-tissue facial asymmetry. Orthognathic surgery brings about a dermatic change, as soft tissue covers underlying bones. Limited soft-tissue asymmetry, meanwhile, is difficult to correct. The treatment modalities for the creation or restoration of an esthetically pleasing appearance were autogenous fat grafts, cartilage graft, and silicon injections. A young female patient had right-side facial asymmetry. The clinical assessment involved visual inspection of the face and palpation to differentiate soft tissue and bone. Although the extra-oral examination found facial asymmetry with skin atrophy, the radiographic findings revealed no mandibular atrophy or deviation. She was diagnosed as localized scleroderma with muscle spasm. In conclusion, facial asymmetry patients with skeletal asymmetry can be esthetically satisfied by orthognathic surgery; however, facial atrophy patients with skin or subdermal tissue contraction need treatment by cosmetic dermatological surgery and orthodontic correction.

• The Journal of Korean Medicine
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• v.36 no.4
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• pp.104-113
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• 2015

A 56-year-old Korean female patient developed generalized morphea over three months. We focused on imbalance of the neuroendocrine-immune axis, mainly using Jiawei-Renshengyangrong-tang and Jiawei-Siqi-tang. Moxibustion on LU9 was conducted for skin symptoms. The patient's modified localized scleroderma skin severity index score decreased from 22 to 6 over 22 weeks of treatment and to 4 during a 3-month follow-up. Her verbal rating scale score for general symptoms decreased from 17 to 1 during the 22-week treatment.

• Annals of dermatology
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• v.30 no.6
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• pp.708-711
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• 2018

Although a few reports have noted the concurrent presentation of morphea and vitiligo at distinctly separate sites in the same patient, it is extremely rare that these two conditions occur at the same sites in a patient. We report the case of a 10-year-old Korean girl with morphea and vitiligo and those lesions occurred at the same sites and progressed simultaneously. An autoimmunity and a cutaneous mosaicism was considered to be involved in such an unique presentation as the pathogenesis is concerned.

• Clinical and Experimental Pediatrics
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• v.58 no.9
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• pp.354-357
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• 2015

Parry-Romberg syndrome (PRS) is a rare, acquired disorder characterized by progressive unilateral facial atrophy of the skin, soft tissue, muscles, and underlying bony structures that may be preceded by cutaneous induration. It is sometimes accompanied by ipsilateral brain lesions and neurological symptoms. Here we present the case of a 10-year-old girl with right-sided PRS and recurrent monoplegic ataxia of the left leg. At 4 years of age, she presented with localized scleroderma over the right parietal region of her scalp; her face gradually became asymmetric as her right cheek atrophied. Brain magnetic resonance imaging revealed hemiatrophy of the face and skull base, and T2-weighted images showed increased signal in the right hemipons and hemicerebellar peduncle. Magnetic resonance angiography findings were unremarkable. She was treated with oral prednisolone, and her recurrent gait ataxia diminished within 2 months of the follow-up period. To the best of our knowledge, this is only the second case of PRS presenting with an abnormal involvement of the ipsilateral hemipons.