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。     

脓疱疹表现的白塞病临床分析

白塞病（BD）是一种原因不明，以细小血管炎为病理基础、呈慢性进行性反复发作的多系统损害疾病，主要表现为复发性口腔溃疡，同时伴有复发性生殖器溃疡、眼损害，皮肤损害为结节红斑样损害、毛囊炎样损害．我科最近诊治了1例白塞病患者，表现为口腔和生殖器溃疡．     

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。     

白塞病的血管损害与免疫学改变

目的：探讨白塞病（ＢＤ）的血管损害以及免疫学改变。方法：对４５例ＢＤ患者的临床及病理学资料进行分析。结果：１４例皮肤组织活检、１例扁桃体活检及１例食道粘膜活检病理改变均符合小血管炎症。２例（２／１２）皮肤直接免疫荧光（ＤＩＦ）阳性，为真皮小血管壁ＩｇＭ和Ｃ３ｃ沉积。４例并发心血管系统损害，其中２例为冠状动脉瘤伴血栓形成。１例为脑栓塞性舞蹈病，１例为食道下段溃疡并出血。有多项免疫学指标异常。１０例检     

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。     

皮肤病综合征

20 0 12 664　白塞氏病 35例临床分析 /李楠 (广东省医院皮肤科 )∥实用医学杂志 .- 2 0 0 1,17( 1) .- 70～ 71男 2 1例、女 14例 ,年龄 4～ 67岁。 68.60 %以口腔溃疡为首发症状 ;74 .30 %以外阴溃疡为首发症状 ;有眼部损害者占 31.4 0 % ;皮肤损害为首发症状 8例 ,为多形性 ;57.14 %针刺反应阳性 ;关节损害为 4 2 .85% ;8例出现心脏损害 ,主要表现为主动脉瓣关闭不全 ,4例出现血管炎损害。作者强调 ,白塞病可能发生心脏损害 ,且可以是本病的主要死因。参 6　 (马勇 )2 0 0 12 665　白塞病与 EB病毒感染及干扰素治疗观察/马百芳 (山东德…     

白塞病合并肠瘘1例

白塞病是一组以反复口腔，外生殖器溃疡，虹膜炎及皮肤损害为特征的多系统损害综合征，以小血管炎为病理基础，其发病机制尚不清楚，白塞病累及多系统的特点使其临床表现复杂多样，我科收治白塞病合并肠瘘1例，现报告如下。     

白塞病30例临床分析

白塞病是一种累及多种脏器的慢性血管性疾病,主要表现为反复发作的口腔外生殖器溃疡,眼部及皮肤损害为特征,可累及全身多个系统的慢性疾病。临床表现多样,诊断上无特异指标,导致其误诊率较高。临床资料统计了2000~2005年上海华山医院住院白塞病30例,其中男21例,女9例,发病年龄22~60岁,平均41岁,20~40岁17例(57%),病程15天~20年,平均6.1年。诊断标准:本组病人的诊断与分型均符合1990年白塞病的国际诊断标准与分型1。本组资料中完全型8例,不完全型22例。临床表现:以口腔溃疡为首发症状者共25例(83.3%),本组病例均发生口腔溃疡。其溃疡可发生于…     

白塞病患者口腔溃疡处血链球菌DNA检测结果分析

目的：检测白塞病患者口腔溃疡组织标本内是否存在血链球菌DNA。方法：8例白塞病患者，8例阿弗他溃疡患者和5例正常口腔粘膜对照进入本研究。采用原位巢式多聚酶链式反应检测口腔溃疡标本是否存在血链球菌DNA。采用限制性片段长度多态性分析对扩增片段进行特异性确证。本研究还对15例白塞病血标本进行了巢式多聚酶链式反应检测。采用四格表资料的确切概率法进行统计学分析。结果：8例白塞病患者口腔溃疡标本中，5例患者血链球菌DNA的表达阳性。8例阿弗他溃疡患者口腔溃疡标本中，2例阳性。5例正常人口腔粘膜全部阴性。经统计学分析：白塞病组与阿弗他溃疡组有统计学差异（P=0．0343）；白塞病组与正常对照组有显著统计学差异（P〈0．01）。15例白塞病血标本全部阴性。结论：白塞病患者口腔溃疡中存在血链球菌DNA，提示血链球菌慢性感染可能与白塞病发病相关。     

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。     

Behçet's disease with relapsing cutaneous polyarteritis-nodosa-like lesions, responsive to oral cyclosporine therapy

Cutaneous polyarteritis-nodosa-like lesions are rarely described in Behcet's disease. We report a case of recurrent cutaneous polyarteritis-nodosa-like (C-PAN-like) lesions in Behcet's disease with multiple deep vein thromboses as part of systemic vasculitis. The mucocutaneous manifestations responded to prednisolone; however, C-PAN-like lesions were refractory and responded to oral cyclosporine. We conclude that C-PAN-like lesions may be marker of severe disease and require intense immunosuppressive therapy.     

Behcet病55例皮肤损害的组织病理学观察

对Behcet病的45例结节性红斑样损害、5例毛囊炎样损害和5例Sweet病样损害的皮肤组织病理学进行观察。除Sweet病样损害伴表皮病变外,其余损害病变主要在真皮网状层和皮下脂肪组织内。主要为毛细血管和细静脉炎。毛囊炎损害为毛囊周围炎,与结节性红斑样一样,伴脓疡样表现。均以淋巴细胞浸润为主。指出这种血管炎既不同于淋巴细胞性血管炎,也不同于白细胞破碎性血管炎。     

Sterile neutrophilic folliculitis with perifollicular vasculopathy: a distinctive cutaneous reaction pattern reflecting systemic disease

The authors prospectively encountered skin biopsies from 20 patients which demonstrated a neutrophilic or suppurative and granulomatous folliculitis accompanied by a folliculocentric neutrophilic vascular reaction of Sweet's-like or leukocytoclastic vasculitis subtypes. While in each case the histomorphology raised diagnostic consideration of bacterial folliculitis, patients frequently expressed systemic complaints such as arthritis, fever, and malaise, and special stains for micro-organisms were negative. Among the clinical presentations were folliculitis, vasculitis, acneiform eruptions, vesiculopustular lesions, and erythema nodosum-like lesions, with the legs, arms, and upper back being the most commonly involved sites. Nineteen patients were found to have specific underlying systemic diseases, namely, inflammatory bowel disease, Reiter's disease, Behcet's disease, hepatitis B, connective tissue disease including mixed connective tissue disease and rheumatoid arthritis, scrofuloderma, and hematologic dyscrasias. The other patient had antecedent bacterial sinusitis in the setting of atopy. The folliculocentric nature of these lesions may reflect preferential processing of antigens through the hair follicle and/or homology between bacterial and follicular heat shock proteins in the susceptible host, namely, one who responds excessively to exogenous antigenic triggers. Folliculitis with folliculocentric vasculopathy may be a clue to underlying systemic disease and/or an extracutaneous infection. Certain light microscopic features in concert with the clinical presentation may distinguish such cases from conventional infectious folliculitis.     

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.     

Management of necrotizing vasculitis with colchicine. Improvement in patients with cutaneous lesions and Behcet's syndrome

Six patients with necrotizing vasculitis were treated with oral colchicine as part of an open study. Four patients with cutaneous vasculitis and normal levels of serum complement and one patient with vasculitis associated with Behcet's syndrome demonstrated clinical improvement while receiving colchicine. One patient with cryoglobulinemia, hypocomplementemia, and cutaneous vasculitis showed no response to colchicine therapy. In three patients, clinical improvement persisted after its withdrawal. Colchicine may be effective in controlling cutaneous necrotizing vasculitis and Behcet's syndrome through its effect on polymorphnuclear leukocyte function.     

Chromosome and sister chromatid exchange studies in Behcet's patients

Behcet's disease is a chronic multisystemic disease of unknown pathogenesis characterized by four major symptoms: oral aphthous ulcers, skin lesions, ocular symptoms and genital ulcerations. The disease is spread throughout the world, but it is most frequent in Turkey, Japan, Korea and China. Although HLA-Bw51 has been found to predominate in Behcet's cases, the genetic etiology has not yet been clarified. In this study, we investigated the chromosomal abnormalities and sister chromatid exchange rates in patients with Behcet's diseases. Thirty-eight patients with Behcet's disease (diagnosed for the first time) and 30 healthy subjects (as controls) were included in this study. Although numerical and structural chromosomal abnormalities were not detected in our patients, we found an increased rate of sister chromatid exchange in patients over the control groups (P < 0.01). On the basis of these results, we discuss the genetic etiology of Behcet's disease.     

Chronic urticaria-like lesions in systemic lupus erythematosus. A review of 12 cases.

Twelve of 54 patients with systemic lupus erythematosus (SLE) had nonpuric, chronic urticaria-like lessions. Skin biopsy of the lesions was performed in 11 cases, and nine showed necrotizing vasculitis. The 54 patients, in general, had severe disease, and laboratory and clinical data suggested a postivite relationship between the urticaria-like lesions and disease severity. There was no consistent relationship between the course of the urticaria-like lesions and the serologic findings and clinical activity of the SLE. The frequency and importance of urticaria-like lesions in SLE deserve further study.