전신성 공피증의 식도내압소견

최명규,박수헌,양진모,조철수,김재광,김호연,정인식,선희식,김부성 대한내과학회 1992 대한내과학회지 Vol.42 No.1

저자들은 1989년 9월부터 1990년 8월까지 가톨릭의과대학 부속병원에서 내원한 전신성 공피증 환자에서 식도내압검사를 시행하여 다음 성적을 얻었다. 1) 대상은 American Rheumatism Association(ARA) 기준에 부합되는 전신성공피증환자로 16세부터 68세(평균 39.2세)의 13명(남 1명, 여 12명)이었고 이 중 2명은 CREST 증후군이었다. 대조군은 17세에서 67세(평균 35.7세)로 정상인 10명(남 1명, 여 9명)이었다. 2) 평균 이환기간은 3.5년±3.7년이고 위장관 증상은 연하곤란 23%(3/13), 속쓰림 23%(3/123), 신트림 15%(2/13), 흉통 7%(1/13), 오심 7%(1/13)순이었다. 전신증상은 Raynaud씨 증상(91.7%)과 근염 30%에서 관찰되었다. 모세혈관확장증과 석회침착증은 CREST 증후군환자(2/13)에서만 발견되었다. 심장침범은 8.4%에서 동반되었다. 3) 하부식도괄약근압은 대조군 24±7.24㎜Hg에 비해 공피증환자군은 1071±4.48㎜Hg로 현저히 저하되어 있었다(p<0.0001). 4) dry swallow시 하부식도수축압은 대조군 43.7±14.7㎜Hg에 비해 공피증환자군은 20.9±23㎜Hg으로 저하되어 있었고(p<0.005), wet swallow시 하부식도수축압은 대조군 64.1±15.2㎜Hg에 비해 공피증환자군은 30.7±24.9㎜Hg으로 저하되어 있었다(p<0.005). 5) 공피증환자군에서 정상식도내압검사인 환자는 13명 중 2례였고, 이상소견을 보인 환자 11명 중 연하곤란과 속쓰림 등의 뚜렷한 임상증세를 가진 환자는 3명에 불과하였다. 이상으로 전신성공피증환자에서 식도운동이상을 시고내압검사로 평가하였다. 84%의 공피증환자에서 이상소견이 확인되엇으며, 공피증의 식도침범에서는 식도증상보다 식도내압소견이 이상이 선행하므로 식도내압검사가 공피증의 식도침범에 대한 진단에 예민하고 정확한 검사로 생각된다. In systemic scleroderma, serious gastrointestinal involvement is present in approximately 50% of the patients. Management of esophageal manifestation of scleroderma is an important factor in the care of the patients of this disease. In the present study, history and symptoms were assessed using a designed questionnaire. Radiologic examination, echocardiographic examination, and skin biopsy were performed for evaluation of systemic manifestations of scleroderma. Esophageal function was assessed in 13 patients with systemic scleroderma by intraluminal manometry and compared with the results from 10 healthy subjects. The results were as follows: 1) All patients with systemic scleroderma were diagnosed based on the diagnostic criteria of the American Rheumatism Association (ARA). The mean age of the systemic scleroderma group was 39.2 years (range 16~68 year) and the male-to-female ratio was 1:9. 2) The mean duration of the disease was 3.5 years (rang 0~10 years) in systemic scleroderma patients. The most common symptoms of scleroderma esophagus were heartburn (3/13) and dysphagia (3/13). Other symptoms such as retrosternal pain, belching, and nausea were observed. It is remarkable that 8 out of 11 patients with systemic scleroderma were asymptomatic in spite of manometrically-proven motor abnormalities. Associated systemic manifestations were Raynaud's phenomenon (91.7%), sclerodactyly (91.7%), arthritis (70%), myositis (30%), telangiectasia (15%), calcinosis (15%), and cardiac involvement (8.4%). 3) Lower esophageal pressure was significantly decreased with a mean value of 10.71±4.48㎜Hg compared to the normal controls with 24±7.24㎜Hg (p<0.0001). 4) Contraction wave amplitudes of the lower esophageal body were decreased in systemic scleroderma with a mean value of 30.7±24.9㎜Hg compared to the normal controls with 64.1±15.2㎜Hg (p<0.001). Simultaneous contraction was observed in 7 patients of systemic scleroderma. Esophageal manometric abnormalities were present in four-fifths of the patients with systemic scleroderma. Since there is a great discrepancy between esophageal symptoms and objectively-proven esophageal involvement, manometry should be used in the assessment of patients with systemic scleroderma.

소아 국소성 경피증의 임상적 고찰

은철,이창우 ( Ehul Eun,Chang Woo Lee ) 대한피부과학회 1997 大韓皮膚科學會誌 Vol.35 No.5

Background: Localized scleroderma, a rare autoimmune disease, can be classified into three subtypes.linear scleroderma, morphea and generalized morphea. Localized scleroderma is usually limited to the skin and subcutaneous tissue but may result in permanent functional disability. Because of its rarity, there are few large scale clinical studies of pediatric patients with localized scleroderma in Korea. Objective : In this study we have examined 11 Korean pediatric patients with localized scleroderma registered at Hanyang university hospital. A review of the clinical presentation, laboratory data, therapy and complcations was carried out in detail. Methods : The medical records of 11 pediatric patients with localized scleroderma seen at the department at Hanyang University Hospital, between 1990 and 1996, were reviewed. The diagnoses were classified into one of the above three localized scleroderma subtypes based on the results of the physical examination, and all patients were examined periodically. The clinical features of the disease, laboratory results, treatment modalities, and complications were summerized from the review of the records. Results : Among the 11 patients, 8 were female and 3 were male; 7 had morphea and 4 had linear scleroderma. The average age at onset was 8.6 years. Antinuclear antibodies above the titer of 1:40 were present in 36%. One of 3 patients with linear scleroderma which involved the upper extremities had moderate degrees of joint contracture. Ten of 11 patients were treated with intralesional injection of corticosteroid and oral medications of hydroxychloroquine, prednisolone and/or nosteraidal anti-inflammatory drugs. Conclusion : Localized scleroderma has no recognized internal organ involvements and is not thought to progress to systemic sclerosis. Since localized scleroderma may cause complications from local atrophy to joint contractures, patients will need to take adequate treatment. and fol- low-up examinations. (Korean J Dermatol 1997;35(5): 877-882)

A case of localized scleroderma with multiple lymphatic vessel dilatation in histopathologic assessment

( Ki-hun Kim ),( Joong-woon Choi ),( Ye-jin Lee ),( Bark-lynn Lew ),( Woo-young Sim ) 대한피부과학회 2019 대한피부과학회 학술발표대회집 Vol.71 No.1

Localized scleroderma is a connective tissue disease characterized by sclerosis of the skin. In histopathologic assessment, localized scleroderma usually showed thickened dermis with sclerotic collagen bundles separated by mucin. Here, we report a rare case of localized scleroderma with multiple lymphatic vessel dilatation in histopathologic assessment. A 33-year old woman presented with a hard skin lesion accompanied tenderness on the right elbow for 1 year. She treated with laser therapy without improvement. Physical examination revealed localized, ill demarcated, skin colored, hard skin lesion on the right elbow. Histopathologic findings revealed thickened dermis with collagen bundles. In lower dermis, there were many of lymphocytes infiltration with multiple lymphatic vessel dilatation which stained by D2-40. Based on these findings, she was diagnosed as localized scleroderma. She treated with topical steroid, with outpatient follow up. In systemic scleroderma, there were several cases about association with lymphangiectasia which showed lymphatic vessel dilatation in the papillary dermis. It is difficult to prove exact mechanism, just as systemic sclerosis. However, this case differs from the previous case in that it showed mid dermis lymphatic vessel proliferation and developed under localized scleroderma, not systemic scleroderma. Here, we report localized scleroderma with mid dermis lymphatic vessel dilation in skin biopsy.

P265 : The effectiveness of UVA-1 phototherapy on localized scleroderma

( Sang Hwa Han ),( Joon Hee Kim ),( Min Soo Jang ),( Kee Suck Suh ),( Sang Tae Kim ) 대한피부과학회 2014 대한피부과학회 학술발표대회집 Vol.66 No.2

Background: Localized scleroderma is a connective tissue disease characterized by thickening and sclerosis of the skin. Recently, studies on the treatment of localized scleroderma with UVA-1 phototherapy has been reported, however there was no previous large study in Asians. Objectives: To investigate the efficacy of UVA-1 phototherapy for localized scleroderma in Asians, and to compare the efficacy of low-dose, medium-dose, and high-dose UVA-1 phototherapy in the treatment of localized scleroderma. Methods: This retrospective study reviewed the response of UVA-1 phototherapy in 19 patients who had been diagnosed with localized scleroderma. Low-dose (20J/cm2), medium-dose (65J/cm2) and high-dose (100J/cm2) regimens of UVA-1 therapy were employed. The therapeutic effectiveness was assessed according to the clinical examintaion before and after the last treatment. Results: Among the 3 cases with low-dose UVA-1 phototherapy, one case showed complete remission (CR) and two cases showed partial remission (PR). Among the 13 cases with medium-dose UVA-1 phototherapy, six cases showed CR and seven cases showed PR. Among the 3 cases with high-dose UVA-1 phototherapy, two cases showed CR and one case showed PR. Conclusion: UVA-1 phototherapy is an effective treatment modality for localized scleroderma in Asians, and high-dose or medium-dose UVA-1 phototherapy might show better response than low-dose UVA-1 phototherapy for localized scleroderma.

Development of an algorithm for Detecting Symptom level in patients with Scleroderma

정진형,이기영,김민영,김남선,이상식 한국정보전자통신기술학회 2015 한국정보전자통신기술학회논문지 Vol.8 No.5

In this study, locality of scleroderma was detected. Diagnostic method is difficult for scleroderma (skin curing; Scleroderma), and it is done by comparing the images of the normal subjects to the scleroderma patients, after performing monochrome processing. The saturation, brightness, and contrast are adjusted, and they were converted by using the process of Well Filter. As a result, the images were able to be used to clearly distinguish the symptoms of scleroderma. In addition, in a video of a healthy person, the line of sight of the observation given the image of scleroderma patients above sea level of height as 0 °is to implement the closing process to the rear Well Filter even only in so that the horizontal plane, and out at intervals of graph the amplitude difference of the video have I asked. The diagnostic criteria were determined for the healthy subjects and the scleroderma patients.

Periostin in Mature Stage Localized Scleroderma

( Min-woo Kim ),( Jung Tae Park ),( Jung Ho Kim ),( Seong-joon Koh ),( Hyun-sun Yoon ),( Soyun Cho ),( Hyun-sun Park ) 대한피부과학회 2017 Annals of Dermatology Vol.29 No.3

Background: Periostin is a novel matricellular protein expressed in many tissues, including bone, periodontal ligament, and skin. Although its expression is prominent in various fibrotic conditions, studies of periostin in localized scleroderma are rare. Objective: To investigate the expression of periostin and other molecules in localized scleroderma. Methods: A retrospective study of 14 patients with confirmed mature stage localized scleroderma was undertaken. Fourteen age-matched and biopsy site-matched subjects with normal skin were included as controls. Collagen fiber deposition, periostin, procollagen, transforming growth factor-β, and matrix metalloproteinase (MMP)-1 expression were assessed and compared between the two groups. Co-localization of α-smooth muscle actin and periostin was evaluated using confocal microscopy. Results: Periostin was predominantly expressed along the dermo-epidermal junction in the controls. Conversely, patients with localized scleroderma demonstrated increased collagen fiber deposition and periostin expression that was more widely distributed along the entire dermis. MMP-1 staining showed increased expression in the epidermis and dermis of patients compared to scanty expression in the controls. A semi-quantitative evaluation showed a higher proportion of excessive collagen bundle deposition (57.1% vs. 7.1%, p=0.013), diffuse periostin positivity (42.9% vs. 0%, p=0.016), and moderate MMP-1 positivity (71.4% vs. 7.1%, p=0.001) in patients than in the controls. Conclusion: Compared to the controls, patients with localized scleroderma had enhanced periostin expression corresponding to increased collagen fiber deposition and unexpected overexpression of MMP-1. The results of this human in vivo study may implicate the pathogenesis of localized scleroderma. (Ann Dermatol 29(3) 268∼275, 2017)

전신성 경피증 피부섬유아세포에서 VII형 교원질 mRNA의 검출

이규석 ( Kyu Suk Lee ),신문석 ( Moom Seok Sihn ),권호준 ( Ho June Kwon ),송준영 ( Joon Young Song ) 대한피부과학회 1996 大韓皮膚科學會誌 Vol.34 No.4

Background: Type VII collagen is a relatively low abundance extracellular matrix protein among the collagenous molecules. Among the minor collagens. type VII collagen has been demon strated by a immunolocalization studies to be component of anchoring fibrils and structures extending perpendicularly from the lamina densa to the upperpapillary dermis. Objective : The purpose of his study is to determine the expression of the type VII collagengene in a group of scleroderma patients as compared to normal skin. Methods : We have examined the levels of type VII collagen mRNA using quantitative reverse transcription PCR and in sit gybridization in scleroderma skin fibroblasts. Immunofluorescent staining with anti-type VII collan antibody was performed in vitro and in vivo to evaluate the expression of type VII collagen at protein level. Results : 1. the ratio of type VII collagen/GAPDH RT-PCR product were 63.3+15.3 in scleroderma and 21.7+7.6 in normal fibroblasts by RT-PCR. 2. The expression of type VII collagen mRNA was considerably lower than type I in scleroderma. A few positive signals by in situ hybridization with type VII collagen cDNA were shown in the dermis. 3. The staining was markedly enhanced in scleroderma fibroblasts and tissues compaired with normal subjects in imunofluorescent staining with anti-Type VII collagn antibody. Conclusion : RT-PCR and immunofluorescent staining with antibodies to type VII collagen shows enhanced gene expression in scleroderma skin fibroblasts. These data suggest that type VII collagen may be the main soruce of the sclerotic change of skin in scleroderma. (Kor J Dermatol 1996;34(4) : 591~599)

Development of an algorithm for Detecting Symptom level in patients with Scleroderma

Jeong, Jin-Hyeong(정진형),Lee, Ki-Young(이기영),Kim, Min-yeong(김민영),Kim, Nam-Sun(김남선),Lee, Sang-Sik(이상식) 한국정보전자통신기술학회 2015 한국정보전자통신기술학회논문지 Vol.8 No.5

본 연구에서는 피부 경화증 환자의 증상정도 알고리즘을 개발하였다. 진단 방법은 피부경화증을 흑백처리 한 후 정상인의 이미지와 비교하였다. 채도, 밝기 및 콘트라스트 조정의 필터를 프로세스를 통해 변환 하였다. 그 결과 화상이 선명한 경화증의 증상을 구별하는데 사용될 수 있었다. 건강한 사람의 영상에서 경화증 환자의 이미지를 주고 폐쇄 프로세스를 적용하여 진폭의 차이로 정상인과 피부 경화증 환자를 결정하였다. In this study, locality of scleroderma was detected. Diagnostic method is difficult for scleroderma (skin curing; Scleroderma), and it is done by comparing the images of the normal subjects to the scleroderma patients, after performing monochrome processing. The saturation, brightness, and contrast are adjusted, and they were converted by using the process of Well Filter. As a result, the images were able to be used to clearly distinguish the symptoms of scleroderma. In addition, in a video of a healthy person, the line of sight of the observation given the image of scleroderma patients above sea level of height as is to implement the closing process to the rear Well Filter even only in so that the horizontal plane, and out at intervals of graph the amplitude difference of the video have I asked. The diagnostic criteria were determined for the healthy subjects and the scleroderma patients.

The Therapeutic Effects of Exosomes Derived from Human Umbilical Cord Mesenchymal Stem Cells on Scleroderma

Yu Yue,Shen Liangliang,Xie Xiaoyun,Zhao Jingjun,Jiang Miao 한국조직공학과 재생의학회 2022 조직공학과 재생의학 Vol.19 No.1

BACKGROUND: Scleroderma is a multisystem disease in which tissue fibrosis is caused by inflammation and vascular damage. The mortality of scleroderma has remained high due to a lack of effective treatments. However, exosomes derived from human umbilical cord mesenchymal stem cells (HUMSCs)-Ex have been regarded as potential treatments for various autoimmune diseases, and may also act as candidates for treating scleroderma. METHODS: Mice with scleroderma received a single 50 lg HUMSCs-Ex. HUMSCs-Ex was characterized using transmission electron microscopy, nanoparticle tracking analysis and nanoflow cytometry. The therapeutic efficacy was assessed using histopathology, immunohistochemistry, immunofluorescence, quantitative real-time polymerase chain reaction, enzyme-linked immunosorbent assay and western blot. RESULTS: HUMSCs-Ex ameliorated the deposition of extracellular matrix and suppressed the epithelial-mesenchymal transition process, and the effects lasted at least three weeks. In addition, HUMSCs-Ex promoted M1 macrophage polarization and inhibited M2 macrophage polarization, leading to the restoration of the balance of M1/M2 macrophages. CONCLUSION: We investigated the potential antifibrotic and anti-inflammatory effects of HUMSCs-Ex in a bleomycininduced mouse model of scleroderma. So HUMSCs-Ex could be considered as a candidate therapy for scleroderma.

A case of localized scleroderma clinically improved with narrowband UVB therapy and colchicine

( Hye Ree Park ),( Young Jun Woo ),( Ji Hee Jung ),( Jung Eun Kim ),( Hoon Kang ) 대한피부과학회 2018 대한피부과학회 학술발표대회집 Vol.70 No.1

Localized scleroderma is a connective tissue disease characterized by sclerosis of the skin. There are currently no curative treatments for scleroderma. Current treatment options include immunosuppressants, anti-fibrotic agents, topical vitamin D, and phototherapy. Recently, UV irradiation has been reported to suppress cellular immunity and also has anti-fibrotic effects. And colchicine has been shown to interfere with collagen synthesis and is used successfully in scleroderma. Herein, we report a case of localized scleroderma improved by narrowband UVB therapy with colchicine. A 53-year-old man presented with asymptomatic, erythematous, thick swelling on the posterior neck for ten years. He didn’t have any other systemic symptoms. He had a history of diabetes mellitus and hypertension. The histopathologic finding showed thickened dermis with sclerotic collagen bundles separated by clear spaces with mucin. Alcian blue staining revealed mucin deposits between collagen bundles in the papillary dermis. Based on the clinical and histological findings, he was diagnosed as localized scleroderma and underwent narrowband UVB phototherapy and colchicine. Narrowband UVB was irradiated with an initial dose of 300mJ/㎠ weekly, which was gradually increased to a maximal dose of 1500mJ/㎠ and colchicine was administered 1200 mg/day. The lesion showed remarkable improvement after ththree years of treatment.