白塞病18例临床分析

白塞病是一种累及皮肤黏膜、眼、滑膜、血管、肠道、肺和神经系统等多系统、多器官的血管炎症性疾患。笔者对１９９１年１月～２００１年１２月１８例住院白塞病患者进行了回顾性分析。１临床资料１．１诊断标准依据１９９０年国际白塞病小组制定的诊断标准[１]。全部病均符合白塞病的诊断标准。其中５例为完全型白塞病，１３例不全型白塞病。１．２一般情况１８例患者男女各９例，年龄４～６５岁，病程２周～８年，病年龄３～６０岁，２０～４５岁之间发病１３例，儿童期发病３例，病年龄最早为３岁，最晚为６０岁。１．３临床器官受累情况…     

52例白塞病回顾性临床分析

目的：探讨我国南方地区白塞病（BD）的可能诱因和临床特点。方法：回顾性分析52例白塞病的临床和实验室资料。结果：52例BD中7例合并结核感染，3例合并肝吸虫感染。男女之比为1．4：1，平均年龄35．4岁，平均病程4．4年。BD可累及多个器官系统，各受累部位的频率依次为：口腔100％，生殖器88．5％，皮肤69．2％，眼40．4％，关节38．5％，呼吸系统5．8％，神经系统5．8％，心脏3．8％，血管1．9％。针刺反应阳性率为65．3％。比较两性在各器官系统受累频率上的差别，发现两性除在生殖器溃疡有显著差异外，余无差异。结论：BD可累及全身多个系统，极易误诊，重要脏器受累预后不良。糖皮质激素或联合应用免疫抑制剂有较好的临床疗效。     

17例白塞病临床回顾分析

目的总结和分析白塞病的临床及治疗特点，以加强对白塞病的认识，提高临床诊治水平．方法回顾性分析17例白塞病患者初发部位、临床表现、实验室和病理检查及治疗的情况。结果本组白塞病以男性多见，好发于（20～50）岁，大部分患者以口腔溃疡为首发表现（70.59％），口腔溃疡的发生率为100％，生殖器溃疡为58．82％，眼部损害为29．41％，针刺反应阳性率为47.06％，皮肤损害以结节性红斑最多见（52．94％），关节受累41．18％，除此外，白塞病尚可累及消化道和神经系统。实验室检查无特异性，病理检查符合血管炎改变，应用激素、免疫抑制剂或联合治疗，所有病例病情均缓解。结论白塞病的诊断主要依靠临床症状．反复口腔溃疡是诊断白塞痛的主要依据．针刺反应有辅助诊断意义．白塞痛治疗目前尚无统一方法，糖皮质激素、免疫抑制剂等治疗能很好地控制病情。     

白塞病64例临床分析

目的：探讨白塞病的临床特点及实验室改变。方法：对64例BD患的临床及实验室资料进行统计分析。结果：口腔溃疡61例,外生殖器溃疡44例,眼部损害21例,皮肤损害33例,针刺反应阳性39例。首发症状以口腔溃疡最常见（42例）。6例并发心血管系统损害,5例并神经系统损害,1例并急性肾功能衰竭,1例并食道下段溃疡并出血;实验室检查有多项免疫学指标的异常,12例检测到自身抗体（12／40）;皮肤及粘膜组织活检病理改变均符合小血管炎症。结论：白塞病是累及全身多系统器官以血管炎改变为基础的免疫性疾病,皮质类固醇激素或激素并免疫抑制剂治疗有较好的临床疗效。     

脓疱型银屑病52例回顾性分析

脓疱型银屑病(PP)是一种少见类型，国内患病率占银屑病的0．56％。本文对本所1986年以来收住院治疗、资料完整的52例PP进行分析，现总结分析如下。1．1一般资料52例中，男35例，女17例，12岁以下9例，13～18岁16例，19～40岁13例，40岁以上14例。有寻常型银屑病史(PSO^ )16例，无寻常型银屑病史(PSO^ )36例。有家族史12例。住院时首     

白塞病的治疗进展

白塞病是一种慢性血管炎性疾病,是口、外生殖器溃疡和虹膜炎三联综合征,也可出现多系统病变,本文介绍了白塞病的发病机理,临床表现,诊断及治疗等方面的情况。     

伴有神经系统症状的白塞病1例

患者男性，２８岁，有反复口腔、阴囊疼痛、溃疡病史，有胸背部瘢痕性痤疮、小腿结节性红斑史，确诊为白塞病６年。３年前出现视物模糊、视力急剧下降，右眼失明。此次因突然头痛、失语、右侧肢体活动障碍３h，于２０００年１０月入院。入院时饮水呛咳，吞咽困难，无抽搐。体检：右眼完全失明，左眼眼前光感，完全运动性失语，右鼻唇沟浅，右侧肢体肌力０级，肌张力增高，颈强两横指，克氏征（±），双侧病理反射阳性。头颅CT未见异常。头颅核磁：双侧脑桥、大脑脚、延髓、丘脑异常信号，考虑脱髓鞘病变。血沉２０mm／h，血常规WBC１１．…     

白塞病38例临床分析

目的　探讨白塞病患者的临床表现、实验室检查和治疗的特点 ,从而提高白塞病的诊断水平 ,以减少白塞病的漏诊、误诊。方法　回顾性分析 3 8例白塞病患者的临床资料。结果　本组病例中 ,主要表现为复发性口腔及外生殖器溃疡、皮肤病变及眼炎。其中以口腔溃疡为首发症状者有 17例 (4 4 .74% ) ,在整个病程中 ,口腔溃疡的发生率为97.3 7%。针刺反应阳性率为 71.0 5 %。行ANCA检查的 5例患者 ,有 4例阳性。根据患者病情的轻重分别给予皮质类固醇激素、免疫抑制剂、反应停、氨苯砜等 ,单用或联用。 3例完全缓解 ,其余均部分缓解。结论　反复口腔溃疡、针刺反应是诊断白塞病的主要依据 ;ANCA检查可能会提高白塞病的临床诊断水平。     

白塞病78例临床分析

目的了解白塞病的临床特点。方法回顾性分析78例白塞病患者的诱因、起病类型、初发部位、临床表现、实验室和病理检查情况及其治疗方法。结果白塞病多见于女性,男:女=1:1.44,好发于青壮年(20~45岁),多为慢性起病,27例急性型发病诱因以上呼吸道感染为主(19例,占70.37%),最常见症状依次是口腔溃疡(96.15%)、皮肤损害(78.21%)、生殖器溃疡(42.31%)、眼部损害(37.18%),其余症状相对较少;实验室检查和病理检查无特异性,皮肤针刺反应阳性率52.56%。结论白塞病的诊断主要依靠临床症状,针刺反应有辅助诊断意义。     

白塞病42例临床分析

目的：探讨白塞病（BD）的临床特点。方法：回顺性分析42例BD患者的临床及实验室资料。结果：BD好发于青壮年，多慢性起病，可累及多个器官系统，各受累部位的频率依次为：口腔42例（100％），生殖器29例（69．0％），皮肤28例（66．7％），关节18例（42．9％），眼18例（42．9％），神经系统6例（14．3％），消化道4例（9．5％），大血管3例（7．1％），呼吸系统2例（4．8％），肾脏1例（2．4％），心脏1例（2．4％）。针刺反应阳性32例（76．2％）。皮肤组织活检12例，病理改变均符合皮肤血管炎。随访发现5例死亡个案中2例为BD肠病，1例为BD肺病，1例为BD脑病。结论：BD是一种以血管炎为基础的可累积全身多器官系统的免疫性疾病，重要脏器受累预后不良。皮质类固醇激素或联合应用免疫抑制剂有较好的临床疗效，但亦带来一定的副作用。     

白塞病患者血清瘦素水平的检测及其意义

目的:探讨瘦素在白塞病发病机制中的作用。方法:采用放射免疫分析法对41例白塞病患者及30例正常对照组血清瘦素水平进行了检测。结果:41例白塞病患者血清瘦素水平(7.37±6.53)ng/mL比正常对照组(4.44±2.56)ng/mL,显著升高(P<0.05);活动期白塞病患者比稳定期白塞病患者及正常对照组瘦素水平显著升高(均P<0.01);有内脏受累白塞病患者比无内脏受累白塞病患者及正常对照组瘦素水平显著升高(均P<0.01);有关节受累白塞病患者比无关节受累白塞病患者及正常对照组瘦素水平亦升高(P<0.05、P<0.01);分组比较结果男、女白塞病患者各组与未分组前的结果一致。结论:白塞病患者体内存在瘦素水平的异常,这种异常可能与白塞病的发生与发展有关。     

HLA—B＊5101与白塞病的相关性研究

目的 :　探讨HLA -B 5 10 1等位基因与白塞病之间的相关性。方法 :　应用微量淋巴细胞毒试验检测HLA -A和HLA -B抗原。应用PCR -SSP技术对HLA -B5 1等位基因 (HLA -B 5 10 1～HLA -B 5 10 7)进行分析。 2 0名白塞病患者符合国际白塞病委员会分类诊断标准 ,同时以 30例正常人作对照组。结果 :　在白塞病患者中 ,HLA -B5 1表型频率明显高于对照组 [患者为 6 0 % (12 2 0 ) ,对照组为 16 .7%(5 30 ) ],χ2 =10 .0 4 ,P <0 .0 0 1,校正P值 <0 .0 5 ,相对危险度为 8.98。没有发现其它HLA -A和HLA -B抗原与白塞病相关。在 12例HLA -B5 1阳性的患者中 ,均为等位基因HLA -B 5 10 1,5个HLA -B5 1阳性的对照亦均为等位基因HLA -B 5 10 1。结论 :　等位基因HLA -B 5 10 1与白塞病的易感性相关     

HLA-A等位基因与白塞病临床表现的相关性研究

目的：探讨HLA- A等位基因与白寒病（BD）临床表现的相关性。方法：应用LABType^TN SSO技术（又称序列微珠综合分析实验系统）对42例BD患者及116人正常对照者的HLA- A等位基因进行检测。结果：与正常对照组相比，有毛睫炎样皮损的BD患者中HLA- A＊02等位基因频率明显降低（P〈0．05），有关节、神经系统受累的BD患者的HLA- A＊29等位基因频率均明显增高（均P〈0．01），有反复口腔溃疡家族史的BD患者中HLA- A＊23等位基因频率明显增高（P〈0．05）。结论：HLA-A等位基因频率与BD的临床表现有一定的相关性。     

以急性发热性嗜中性皮病皮疹为表现的白塞病1例

分析报告 1例以急性发热性嗜中性皮病皮疹为表现的白塞病。患者表现为急性发热性嗜中性皮病皮疹 ,伴口腔溃疡、关节疼痛 ,组织病理符合皮肤急性发热性嗜中性粒细胞增多性皮病 ,皮肤针刺反应阳性 ,诊断白塞病。     

Behcet病21例临床分析及眼内超声表现

目的：探讨Behcet病的临床特征以及超声检查对眼部损害的诊断价值。方法：回顾性分析了21例Behcet病的临床资料，包括8例眼部损害患者（13只眼）的超声图像特点。结果：口腔溃疡、外生殖器溃疡、皮肤损害、眼部病变是本组Behcet病的基本临床特征，以口腔溃疡最常见。前房积脓，玻璃体雪球状浑浊，伴粟粒样强回声光点及纤维光带形成等是Behcet病的主要超声表现。结论：某些口腔溃疡反复发作常规治疗效果欠佳时，可能是Behcet病的早期表现。超声检查可对Behcet病眼部损害以及本病的早期诊断提供有用的影像学依据。     

Acute generalized exanthematous pustulosis: A retrospective study of 51 cases in Taiwan

Background/ObjectiveAcute generalized exanthematous pustulosis (AGEP) is a severe cutaneous adverse drug reaction characterized by fever and numerous sterile non-follicular pustules. It is mainly attributed to drugs, although other factors have been implicated. The objective of this study was to evaluate the clinical and histological features of AGEP in a Taiwanese population.MethodsIn this retrospective study, we reviewed patients diagnosed with AGEP with a EuroSCAR (RegiSCAR) validation score more than 4 (>4, probable to definite cases), between 1992 and 2012 at the Chang Gung Memorial Hospital in Taiwan. Demographic, clinical and laboratory data, pathologic findings, and disease causality were analyzed.ResultsA total of 51 patients were included in this study, with 34 (66.7%) patients being diagnosed with AGEP with drug causality, and 17 (33.3%) patients being diagnosed with AGEP without drug causality. Cases of AGEP with drug causality showed an older average age, and a significantly higher rate of previous drug hypersensitivity history compared to cases of AGEP without drug causality (p = 0.0018). None of the patients had a history of psoriasis or had developed psoriasis at the 1-year follow-up. A total of 12 cases (23.5%) had systemic involvement, including liver and kidneys. Penicillin or aminopenicillin (17.6%) and cephalosporins (17.6%) were the most common causative drug groups related to AGEP. In AGEP patients without drug causality, three cases of pathogen infections were identified (1 case of mycoplasma, Coxsackie virus, and Epstein-Barr virus, respectively).ConclusionWe found that beta-lactam antibiotics were the major drug class responsible for inducing AGEP in a Taiwanese population, but that some infectious pathogens may also contribute to AGEP development.     

Oral zinc sulfate in the treatment of Behcet's disease: a double blind cross-over study

This was a randomized, controlled, double-blind trial of zinc sulfate in the treatment of Behcet's disease. Patients with Behcet's disease were recruited in this study between November 2001 and February 2003. A clinical manifestations index (CMI) was calculated for each patient. Serum zinc was estimated in all patients both at the beginning and monthly throughout the trial. Serum zinc levels were estimated from 30 healthy normal subjects matched for age and sex as a control group. Patients were randomly allocated to receive either 100 mg zinc sulfate or identical placebo tablet three times daily in a double-blind manner. After 3 months of starting treatment, patients were crossed over, that is, patients on placebo received zinc sulfate and vice versa. Mean serum zinc level in Behcet's disease patients was statistically significantly lower than mean serum zinc levels in healthy the control. In group A (started with zinc sulfate), the mean CMI started to decline directly after the first month of therapy with zinc sulfate to significantly lower levels. After shifting to placebo treatment in the fourth month, the mean of CMI started to rise again gradually but remained significantly lower than levels before therapy for the fourth and fifth months. In group B (started with placebo), the mean of CMI remained high for the first 3 months. After crossing over to zinc sulfate in the fourth month, the mean of CMI started to decrease after the fourth month. An inverse correlation between CMI and serum zinc level was found. No side-effects were seen in either group. In conclusion, zinc sulfate was found to be a good option in the treatment of Behcet's disease.     

Oxidative stress in patients with Behcet's disease: I correlation with severity and clinical parameters

The study was designed to investigate the possible correlation between some oxidative stress parameters in Behcet's disease and the clinical manifestations of the disease as well as the possible correlation with the disease severity. Seventy-six patients diagnosed according to the International Study Group criteria for Behcet's disease were included in the study. Sixty patients had mild-to-moderate disease and 16 patients had severe disease. Sixty matched control subjects were also included. After a full history and examination from each subject, 10 mL blood was drawn from each for analysis. Serum malondialdehyde, glutathione, ceruloplasmin, copper and zinc levels were determined. Patients with Behcet's disease showed increased levels of serum malondialdehyde and copper while glutathione and zinc levels were decreased. Comparison between these parameters in patients with mild-to-moderate disease with those with severe disease showed only that serum zinc levels were lower in severe Behcet's disease. Serum malondialdehyde levels were found to be significantly positively correlated with oral ulcer size, duration and frequency. Glutathione levels were found to be inversely correlated with the clinical manifestation index and all oral ulcer parameters. Zinc levels were found to be inversely correlated with the clinical manifestation index and pathergy test positivity grades. Copper levels were found to be positively correlated with oral ulcer number. Although the parameters of oxidative stress did not show correlation with disease severity, they were correlated with the disease manifestations. This points out the importance of oxidative stress in Behcet's disease.