• 대한두개안면성형외과학회지
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• 제18권3호
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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.

• 대한두개안면성형외과학회지
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• 제20권2호
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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.

• 대한소아치과학회지
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• 제32권2호
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• pp.256-261
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• 2005

피부 경화증(scleroderma)은 만성 결체조직 질환으로 정확한 원인은 밝혀지지 않았으나 자가 면역 질환으로 알려져 있다. 대개 $30{\sim}50$세의 여성에서 호발하며 소아에서는 매우 드물다. 장기의 이환여부에 따라 국소성과 전신성 피부 경화증으로 분류할 수 있다. 국소적 형태는 예후가 양호하고 피부에만, 또는 피하 근육까지 이환되며, 내부 장기에는 이환되지 않는다. 전신적인 형태는 피부와 구강점막 및 위장계, 호흡계, 심혈관계 등을 포함하늘 전신 다발적 발생 (multisystemic involvement)이 특징적이다. 안면피부 이환시 작고 예리한 코, 무표정한 응시 (expressionless stare), 좁아진 입모양(narrow oral aperture) 등이 특징적이다. 대개 레이노 현상(Raynaud's phenomenon)이 존재하며 질환이 진행되면서 개구장애와 혀, 치은의 경화가 나타나게 된다. 피부 석회증(calcinosis cutis), 레이노현상(Raynaud's phenomenon). 식도 기능부전(esophageal dysfunction), 공지증(sclerodactyly), 모세혈관 확장증(telangioectasia)이 복합된 질환을 크레스트 증후군(CREST syndrome)이라 부른다. 피부 경화증의 치료는 국소적 및 전신적 스테로이드 치료, collagen cross-link inhibitor (D-penicillamine), 면역억제제 등을 사용한다. 개구제한의 치료는 설압자를 이용한 mouth stretching exercise를 통해 개선될 수 있다. 본 증례에서는 국소성 피부 경화증으로 진단되고 개구제한이 있는 6세 남아에게 아산화질소와 enflurane을 이용한 깊은 진정요법을 이용하여 상하악 제 1, 2 유구치의 우식치료 치험례를 보고한다.

• Maxillofacial Plastic and Reconstructive Surgery
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• 제37권
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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.

• 대한한의학회지
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• 제36권4호
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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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• 제30권6호
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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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• 제58권9호
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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.