风湿性心脏病合并系统性硬皮病1例

风湿性心脏病合并系统性硬皮病１例朱俊兰，朱文元，张美华患者女，３７岁，因活动后胸闷气促１２年，咳嗽，咯白色粘液痰３周，全身皮肤渐变硬１０年，加重３周于１９９４年１０月８日入院，患者于１２年前出现活动后胸闷、气促、心悸且逐渐加重，发病后来我院心脏科门诊...     

系统性硬皮病并广泛肌肉钙化1例

患者男,42岁。全身皮肤变硬13年,皮肤、肌肉萎缩7年于1994年4月20日住院治疗。13年前无明显诱因出现右前臂皮肤变硬,逐渐发展至全身。7年前全身肌肉萎缩,伴吞咽、行走及下蹲困难。体检:营养不良,满月脸,全身皮肤呈弥漫性灰褐色及暗红色萎缩变硬,尤以双侧前臂至指端、双小腿至趾     

系统性硬皮病合并肝损伤1例并文献复习

患者女,72岁,主因"面部、双手硬化性斑块10余年,加重1个月余"入院.诊断:系统性硬皮病(SSc).入院后予甲强龙60 mg/d(合泼尼松75 mg/d)静脉滴注抗炎治疗,予桂枝红花汤煎服,出院后激素逐渐减量.     

系统性硬皮病重叠系统性红斑狼疮1例

报告1例系统性硬皮病重叠系统性红斑狼疮。患者女,19岁,面部、双手暗红斑、硬肿半个月,伴四肢关节痛。面部红斑日晒后加重。专科情况:面颊部、口鼻周围淡红斑,呈蝶形分布,伴硬肿,双手指肿胀,呈腊肠样改变,手背皮肤发紧、变硬,握拳受限;双足趾末端稍发绀,皮温略低;双侧手关节、膝关节轻度压痛。辅助检查:尿蛋白(+++),两次24小时尿蛋白定量分别为595.0↑mg/24h、581.9↑mg/24h;免疫5项:C3 0.67↓g/L,C4 0.11↓g/L;自身免疫抗体:ANA定量1∶3200阳性(核仁+粗颗粒),nRNP/Sm-免疫印迹法阳性↑,Sm阳性↑,SS-A强阳性,Ro-52强阳性,SS-B阳性,Scl-70强阳性;组织病理:表皮基底层色素增加,真皮胶原增粗,肿胀,胶原间少许粘蛋白沉积,血管周围少许淋巴组织细胞、个别嗜中性白细胞,脂肪小叶间隔增宽,胶原硬化。诊断为:系统性硬皮病重叠系统性红斑狼疮。     

系统性硬皮病伴发肺癌1例

１病历摘要患者男 ,６０岁 ,双手、前臂及前胸皮肤硬化１４年 ,伴心慌、胸闷３个月。１４年前 ,患者常因寒冷或精神紧张时 ,双手指皮肤苍白、麻木发凉 ,数分钟后皮肤青紫、疼痛 ,随保暖或精神稳定后局部皮肤潮红而恢复正常。冬重夏轻。后双手指及手背逐渐出现非凹陷性肿胀 ,皮肤逐渐紧张、变硬 ,皮纹逐渐消失 ,渐扩至前臂。８年前 ,面部渐变呆板无表情 ,前胸皮肤紧张有束缚感。曾２次以＂系统性硬皮病＂住我科治疗好转出院。近３个月 ,患者觉前胸束缚感加重 ,常咳嗽、咳少量泡沫痰 ,无咳血、血痰及咯血 ,伴胸闷、气急、心慌 ,不能劳累 ,进食…     

系统性硬皮病34例临床分析

系统性硬皮病(PSS)是有广泛的皮肤硬化和多脏器受累的系统性皮肤病。现将我院1986年～1996年收治的34例PSS分析报告如下。一般资料　34例中男7例,女17例,男女之比为1∶3.8。发病年龄10～60岁,<20岁2例,>50岁3例,21～49岁29例,病程2月～20年。其中农民19例,工人7例,其他8例。临床表现　发病多无明确的诱因,其中3例分别在手术后1周,被雨淋发高热后及连续5年双手冻疮,半年未愈后发病。1/3患者以雷诺现象为初发症状,其次为四肢关节痛、双上肢肿胀、麻木,渐发生皮肤变硬。发病期间23例有雷诺现象,其中2例发生指端坏死。18例关节疼痛肿胀,以四…     

皮肌炎合并白癜风1例

患者女，46岁。因面部红斑1年，四肢乏力9个月，全身泛发白斑3个月就诊。1年前无明显诱因鼻根部及双眶周围皮肤出现红斑，伴肿胀及轻微瘙痒；9个月前面部出现红斑，累及整个面部、颈项、胸骨区及前臂屈侧，同时出现四肢乏力。在外院检查示：抗核抗体(ANA)1：1000，IgA3470mg／L。肌肉活检示局灶性肌纤维颗粒变性及空泡变性，符合皮肌炎改变，给予泼尼     

系统性硬皮病105例临床分析

对１０５例系统性硬皮病患的一般情况、临床表现了详细分析。男女患者之比为１：４，发病年龄１５岁－７２岁，２１－５０岁患者占大多数，所有患者均有不同程度的皮肤改变，皮受累范围大小不一。９９％，患者嗓手皮肤受累，８１％患者有雷诺现象，６７例患者内脏器官受累。还对其实验室检查，伴发病、病因、诊断和治疗作了分析和讨论。     

白癜风合并银屑病1例

患者男,79岁.腰背部及臀部皮肤白斑50年,伴鳞屑性丘疹及瘙痒半年.患者50年前腰背部及臀部皮肤出现不规则白斑,逐渐增大,无自觉症状,未进行任何治疗.     

A Case of Progressive Systemic Sclerosis Associated with Sarcoidosis and Esophageal Adenocarcinoma

A 50-year-old Japanese female with progressive systemic sclerosis (PSS, CREST syndrome) is reported. During treatment for PSS, she was diagnosed by clinical and laboratory findings as having sarcoidosis, which was confirmed by histological examination of the skin and lymph nodes in July of 1991. She complained of back pain in August of 1991. Reflux esophagitis and Barrett's esophagus, found by endoscopy, progressed into a well-differentiated tubular adenocarcinoma. This is a very rare case of PSS associated with sarcoidosis and esophageal adenocarcinoma developing from Barrett's esophagus.     

儿童系统性硬皮病1例

患儿女性,2岁8个月。全身皮肤变硬、双手指结节1年余。结合临床表现以及实验室检查确诊为儿童系统性硬皮病。予激素、复方甘草酸苷、青霉素、扩管药物及中草药治疗,取得一定的疗效。     

A Case of Multicentric Reticulohistiocytosis,Systemic Sclerosis and Sjögren Syndrome

We report the case of a 42-year-old Japanese woman who developed multicentric reticulohistiocytosis (MR) complicated by systemic sclerosis (SSc) and Sjögren syndrome (SS). The patient complained of tender nodules on the left hand, polyarthralgia in the finger joints and knees, and xerostomia. The skin nodules were distributed mainly on her hands and fingers with skin sclerosis. The serum anti-nuclear test revealed anti-centromere antibody and the discrete speckled pattern of anti-nuclear antibody. The biopsy specimens from the finger nodule and the sclerotic finger skin showed a perivascular infiltration of multinucleated giant cells with ground-glass cytoplasm and dermal thick collagen proliferation, respectively. The lip biopsy and sialography specimens showed periductal lymphocyte infiltration and apple tree-like changes. Systemic corticosteroid treatment improved the polyarthritis, xerostomia, and skin sclerosis rapidly but suppressed the nodular lesions only gradually. This is the first report of a combined case of MR, SSc and SS. This multiple autoimmune complication suggests the involvement of an immunological disturbance in the pathogenesis of MR.     

Treatment of Systemic Sclerosis

Systemic sclerosis is an extremely variable disease in its manifestations and consequently, treatment needs to be individualized depending on the specific problems that each patient has. Limited scleroderma patients have a prolonged duration of Raynaud’s phenomenon and puffy fingers before they develop any skin thickening, digital ulcers or gastrointestinal symptoms. They are likely to present with all the classic manifestations of scleroderma. Diffuse scleroderma patients have a much more acute systemic onset with marked whole hand swelling and may initially have only subtle skin thickening. A good understanding of the differences between the natural history of limited and diffuse scleroderma will enable the physician to treat present problems and anticipate future ones more effectively. One should determine which major subset and organ systems are involved before deciding on the appropriate therapy. Advances in organ-specific therapy, particularly calcium channel antagonists in Raynaud’s phenomenon, proton pump inhibitors in esophageal reflux, intravenous iloprost and endothelin receptor antagonists in pulmonary hypertension, and ACE inhibitors in renal crisis, have decreased morbidity and mortality in patients with scleroderma. Studies of aggressive therapies to prevent or improve pulmonary fibrosis are in progress. Further clinical experience in wound healing, gastrointestinal malabsorption and physical therapy for loss of motion has helped patients to have a more comfortable life. In recent years, a significant number of controlled clinical trials have been performed and there has been improved understanding of the best way to perform studies and of which patients are most likely to respond to therapy. Penicillamine, methotrexate, photopheresis, relaxin, interferons, and cyclosporine have all been studied in controlled trials with variable outcomes. Although an overall remittive therapy has not yet been determined, new, potentially useful agents are being investigated.     

结节性硬化症1例

1 临床资料 患者,男,21岁,因全身皮疹20年于2009年8月到本中心门诊部诊治.1岁多时无明显诱因面部出现丘疹,躯干四肢出现皮赘,以后逐渐增多,扩大.面部皮损增至米粒大小,呈淡红或暗红色,躯干、四肢皮疹增至黄豆大小或拇指头样斑块,呈肤色或红黄色;3岁时背腰部出现局限性色素脱失斑,无自觉症状.     

Antiphospholipid Syndrome Associated with Progressive Systemic Sclerosis

We report a case of secondary antiphospholipid syndrome (APS) occuring in a progressive systemic sclerosis (PSS) patient who took herbal medication. Clinical findings compatible with APS included positive IgM anticardiolipin antibody (ACL), thrombocytopenia, and obstruction of the left radial artery on digital subtraction angiography (DSA), Clinical findings compatible with PSS included sclerodactyly and digital ulcers, Raynaud's phenomenon, pulmonary fibrosis and pulmonary hypertension, proteinuria and renal mesangial reaction, and myocarditis.     

Diffuse Systemic Sclerosis in a Patient with Primary Biliary Cirrhosis and Autoimmune Hepatitis Overlap Syndrome: A Case Report

Systemic sclerosis (SSc) is a chronic systemic disease of unknown etiology characterized by vasculopathy, excessive accumulation of extracellular matrix, and fibrosis of the skin and other internal organs. Although its etiology remains elusive, approximately one third of SSc patients presents with additional autoimmune disease, which suggests that an autoimmune mechanism is a major component of the underlying pathophysiology. On the other hand, primary biliary cirrhosis (PBC) and autoimmune hepatitis (AIH) are two main autoimmune liver diseases. A 41-year-old female previously diagnosed with PBC/AIH overlap syndrome presented with multiple, painful brownish to erythematous firm patches on the hands, arms, axillae, neck, abdomen, and thighs. Laboratory work-up yielded positive results for anti-nuclear antibody, anti-Ro/Sjögren''s-syndrome-related antigen A autoantibodies, and perinuclear anti-neutrophil cytoplasmic antibodies while punch biopsy of her left hand showed characteristics that are consistent with scleroderma. Herein, we report the first case of a patient with diffuse cutaneous SSc and concurrent PBC/AIH overlap syndrome and suggest that this coexistence of multiple autoimmune diseases is not a coincidence but rather that a common autoimmune pathogenesis may exist.     

系统性硬皮病患者缺氧与皮肤硬化关系的初探

目的 探讨缺氧对系统性硬皮病 (SSc)患者皮肤硬化的影响。方法 测定 SSc患者血氧分压和皮肤硬度积分,对二者进行直线相关分析,并测定低氧 (2.5％氧浓度 )对体外培养的 SSc患者皮肤成纤维细胞增殖的影响。结果 30例 SSc患者血氧分压为 6.38～ 12.06 kPa,平均 (9.53± 1.93)kPa; 20例正常对照组为 (10.67～ 13.33)kPa,平均为 (12.01± 0.81)kPa。前者比后者显著降低 (t=5.276,P