CREST综合征1例

患者女,20岁。因面颈部、躯干、双上肢皮疹,双手指遇冷发白变紫8年于本科就诊。患者8年前于右鼻部近眼内侧皮肤出现小片红色皮疹,渐发展至整个面部、颈部、胸背部、双上肢、双手背及手掌,同时双手皮肤逐渐变硬,遇冷疼痛,尤以冬季为重．易患冻疮。曾口服、外用中药(具体不详),疗效欠佳。患病以来无发热、关节和肌肉疼痛。既往无特殊病史。体格检查：T36．5℃．P72次／min．R20次／min,BP17．3／10．7kPa(130／80mmHg)。     

CREST综合征一例

患者男,45岁.因全身皮疹12年,皮肤变硬6年,加重半年入院.患者于12年前无明显诱因下出现面部、颈部、胸前小片红色不高出皮面皮疹,逐渐增多并累及四肢.6年前,患者面部、手足出现皮肤肿胀,逐渐变硬,手指末端变细,难以捏起,口唇变薄,且双手足遇冷水易发白变紫疼痛,冬季为甚.     

CREST综合征1例

患者女,72岁.肢端硬化、吞咽困难近3年.患者于3年前无明显诱因出现吞咽食物时,尤其是质硬食物时下咽困难,同时双手指较前变硬,患者未予特别重视,也未进行系统检查及治疗.     

CREST综合征发射性计算机断层扫描全身骨显像1例

患者女，32岁，因躯干，四肢皮肤发紧变硬，皮肤结节2年于2003年6月入住我科。患者5年前出现四肢大关节游走性疼痛，2年前双手背，前臂皮肤出现硬肿，很快延及上臂，颜面，上胸部皮肤，伴吞咽不畅，胸部了压迫感，同时双肘关节伸侧及髓关节，腕关节附近皮肤出现硬节，间有溃破，流出乳白色沙砾状物。双手指遇冷后变紫，双腕关节疼痛，3个月前在外院查抗双链（ds）-DNA,     

CREST综合征1例

CREST综合征是系统性硬皮病的一种,临床较少见,近日我科诊治1例,现报道如下.     

抗线粒体Ⅱ型抗体与抗着丝点B蛋白抗体重叠阳性在CREST综合征中的临床意义

采用间接免疫荧光(IIF)法、酶免疫斑点(ELISPOT)法和免疫印迹(Western blot)法检测107例进行性系统性硬皮病(SSc)患者,血清中的ANA阳性率及核型、AMA阳性率及亚型AMA-M2、AMA-M4和AMA-M9的阳性率、CENP-B和Scl-70的阳性率,分析AMA-M2和CENP-B在CREST综合征中的重叠阳性率,同时记录AMA-M2和CENP-B 重叠阳性患者的临床症状和相关实验室指标,探讨AMA-M2与CENP-B重叠阳性在CREST综合征中的临床意义.     

CREST综合征1例

CREST综合征为系统性硬皮病的一种特殊类型,临床少见,笔者将长海医院所见1例,报道如下. 临床资料 患者男,16岁,汉族.因全身散在红斑12年、手指关节变硬8年、臀部结节6年来我院就诊.     

皮肌炎、硬皮病及桥本氏甲状腺炎重叠综合征1例

患者女,２１岁。乏力、肌痛、间断发热,并有皮肤、肌肉萎缩变硬２年。皮肤科检查示肌肉萎缩,皮肤弹性差,Ｒａｙｎａｕｄ（＋）。实验室检查：抗Ｓｃｌ－７０（＋）,抗Ｊｏ－ｌ（＋）,补体Ｃ３、Ｃ４降低,ＣＫ明显高于正常值,Ｔ３降低,ＴＭＡ（＋）,ＴＧＡ（＋）。心电图示Ｔ波改变。肺Ｘ线摄片不除外肺间质改变。肌电图为肌原性萎缩相。皮肤、肌肉病理活检符合硬皮病、皮肌炎改变。     

CREST综合征一例

患者男,45岁.因全身皮疹12年,皮肤变硬6年,加重半年入院.患者于12年前无明显诱因下出现面部、颈部、胸前小片红色不高出皮面皮疹,逐渐增多并累及四肢.6年前,患者面部、手足出现皮肤肿胀,逐渐变硬,手指末端变细,难以捏起,口唇变薄,且双手足遇冷水易发白变紫疼痛,冬季为甚.     

CREST综合征一例

<正>临床资料患者,女,43岁。主因四肢皮肤发硬、下肢结节8年,伴溃烂、疼痛6个月,于2013年9月就诊。8年前无明显诱因患者双下肢皮肤出现肿胀、渗出,小腿出现2个无痛性结节,在外院诊断为硬皮病,经治疗症状好转。此后结节处经常自行破溃,能挤出白色、质硬物质,用药后创面多能好转;患病期间自觉冬季手脚发凉麻木,手指皮肤变硬且活动受限;间断口服糖皮质激素药物6年余,泼尼松量由30 mg     

Olmsted syndrome: report of two new cases and literature review

Olmsted syndrome is a rare keratinization disorder; 18 cases have been published so far. It associates a mutilating cogenital palmoplantar keratoderma with periorificial erythematokeratotic lesions. We report herein two new unrelated male children with Olmsted syndrome (OS), one of whom was studied by light and electron microscopy. Our histological, immunohistochemical, and ultrastructural findings suggest that this disease is related to epidermal hyperproliferation. We present herein a review of the twenty cases published so far and discuss the major clinicopathological and genetic features of this disease.     

Trigeminal trophic syndrome: a report of two cases with review of literature

Trigeminal trophic syndrome, a rare cause of facial ulceration, is the consequence of damage to the trigeminal nerve or its central sensory connections. We report two cases of trigeminal trophic syndrome, complications of cerebral vascular accidents. A 65-year-old woman complained of numerous and progressive ulcers of the right side of her face of 2 month's duration. A 67-year-old woman presented with an ulcer of the ala nasi of 10 week's duration. This syndrome most commonly occurs in women with an average age of 57 years. About 100 cases are reported in the literature. The diagnosis is made on clinical grounds, and is suggested when loss of sensation occurs in association with unilateral facial ulceration, especially involving the ala nasi. Once the ulcers appear, they are extremely persistent.     

毛囊黏蛋白病二例并文献复习

报道2例毛囊黏蛋白病并进行文献复习.患者1,男,44岁,颈项部局限性浸润性红斑伴疼痛2个月,组织病理示:真皮毛囊水肿,毛囊内外淋巴组织细胞浸润,个别嗜酸粒细胞.阿新蓝染色:毛囊黏蛋白阳性.患者2,男,32岁,左面部浸润性暗红斑45天,组织病理示:表皮大致正常,毛囊上皮水肿,淡蓝色黏液样物质沉积,毛囊内及毛囊周围较多嗜酸...     

无水疱性类天疱疮二例并文献复习

报道2例无水疱性类天疱疮并复习以往相关文献。例1，男，72岁，因全身皮肤红斑、丘疹伴瘙痒20天就诊，查体未见水疱。实验室检查血嗜酸性粒细胞升高，直接免疫荧光示基底膜带线状C3沉积，间接免疫荧光示抗基底膜带抗体阳性，抗BP230抗体、抗BP180抗体阳性。例2，女，79岁。因全身皮肤散在分布丘疹伴严重瘙痒20天就诊，查体见躯干四肢皮肤抓痕明显，未见水疱。血清中抗BP180抗体阳性。2例均诊断：无水疱性类天疱疮。予以小剂量糖皮质激素治疗后，控制病情，目前在随访中。     

带状疱疹后假性腹壁疝二例并文献复习

带状疱疹后假性腹壁疝是带状疱疹发生的一种罕见的与腹壁疝相似的神经系统并发症,所引起的腹壁隆起性肿物实际上是病毒引起节段性腹肌瘫痪或无力的结果,大多数可完全恢复,预后良好。本文报道2例带状疱疹后假性腹壁疝并对10年国内外文献报道病例进行分析。共分析27例患者,平均年龄68.2岁,63.0%为男性,常受累的神经是T11（31.4%）。25例腹壁假疝发生在皮肤症状之后,平均时间为23天。行肌电图检查3例,异常2例; 影像学检查15例,其中异常5例。27例中,14例(51.9%)腹壁假疝完全自然康复,平均时间为3.4个月(范围1~8个月);3例(11.1%)部分恢复,平均随访时间2.3个月。7例患者恢复时间不详,1例失访。     

Primary localized cutaneous nodular amyloidosis and CREST syndrome: a case report and review of the literature

Primary localized cutaneous nodular amyloidosis (PLCNA) is a form of primary localized cutaneous amyloidosis (PLCA) that presents as yellowish waxy nodules on the extremities, face, trunk, or genitalia. We report the case of a patient with PLCNA and CREST (calcinosis, Raynaud phenomenon, esophageal motility disorders, sclerodactyly, and telangiectasia) syndrome. A diagnosis of her extensive PLCNA was made after biopsy specimens from the bilateral shins stained positive for amyloid extending from the superficial papillary dermis to the subcutis. Results of a workup were negative for paraproteinemia or signs of systemic amyloidosis and have remained so after 8 years of follow-up. We present a review of the literature describing the presentation and histopathology of the varying forms of amyloidosis.     

肉芽肿性蕈样肉芽肿二例并文献复习

报道2例以局部浸润性斑块为主要表现的肉芽肿性蕈样肉芽肿并复习相关文献.2例患者均表现为红斑、斑块,皮损组织病理检查见真皮内淋巴样细胞及巨细胞浸润,患者1免疫组化示CD2、CD3、CD5、CD7阳性,患者2 CD2、CD3、CD4阳性及CD68组织细胞阳性.诊断:肉芽肿性蕈样肉芽肿.患者1口服阿维A及肿块局部浅层X线照射...     

海分枝杆菌感染二例并文献复习

报道2例海分枝杆菌感染并复习相关文献.患者1,男,25岁.右侧拇指红肿破溃伴疼痛1个月.患者2,女,54岁.左侧大拇指及手背手腕红斑结节伴疼痛2个月.2例患者均有鱼扎伤病史,常规抗感染治疗无效,最终诊断为游泳池肉芽肿,给予抗分枝杆菌感染治疗3个月后痊愈.     

播散性带状疱疹二例报道并文献复习

带状疱疹（HZ）皮损通常限于单个皮区,而涉及两个或更多个皮区的非邻近HZ比较少见,我们报道2例播散性HZ患者并回顾相关文献。病例1,男,77岁。右臀部簇集性丘疹、水疱伴疼痛6天,全身皮疹2天,治疗上给予抗病毒、营养神经和对症治疗,病情得到控制,未见新发皮疹,仍有神经疼痛。病例2,女,32岁。左侧胸背部簇集性丘疹、水疱伴疼痛4天,全身皮疹伴痒1天,因患者处于哺乳期,未使用抗病毒药物,局部涂抹炉甘石洗剂,2周后自愈。     

Accessory tragus: report of two cases and review of the literature

Accessory tragus is a fairly common congenital malformation of the external ear. In the vast majority of cases it is an isolated developmental defect not associated with other abnormalities. However, the remote possibility exists that it could be associated with other abnormalities of the first and second branchial arch. Accessory tragus is a consistent feature of the oculoauriculovertebral syndrome (Goldenhar syndrome). When correctly identified, surgical excision of accessory tragus is quite simple and rarely results in any complications.