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。     

再生障碍性贫血并发白塞病1例

<正>白塞病(Behcet’s disease, BD)是一种病因不明,以血管炎为基础的慢性疾病,临床上以口腔溃疡、生殖器溃疡、眼部病变及皮肤损害为主要表现。该病常累及消化道、中枢神经、肺部、肾脏以及附睾等器官和系统,呈复发和缓解的交替过程[1]。BD的临床表现可因性别、年龄的不同而变化,表明其临床表现的复杂多变[2]。该病     

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。     

经腹与经阴道彩色多普勒超声在早期异位妊娠中的诊断价值的对比研究

目的：探讨经阴道彩色多普勒超声在早期诊断异位妊娠中的临床应用情况。方法：收集本院2011年10月至2012年10月期间经临床确诊的异位妊娠患者90例的临床资料,且该90例患者均在早期进行过经腹部彩超及经阴道彩超检查,对两种早期彩超检查方法的临床诊断准确率情况进行比较性分析。结果：该组患者经腹部彩色多普勒超声检查的临床诊断准确率为77.78％,经阴道彩色多普勒超声检查的临床诊断准确率为96.67％,经阴道彩色多普勒超声检查的临床诊断准确率明显高于经腹部彩色多普勒超声检查（χ2＝14.41,P＜0.05）,有统计学意义。经阴道彩色多普勒超声检查的各种典型异位妊娠超声影像学表现（胚芽、盆腔积液、原始心血管搏动、附件包块、子宫内假孕囊）检出率均显著高于经腹部彩色多普勒超声检查,P＜0.05,有统计学意义。结论：经阴道彩色多普勒超声在早期诊断异位妊娠方面较传统经腹部彩超具有显著性优势,对于有效降低异位妊娠死亡率具有重要意义,值得临床进一步推广。     

白塞综合征128例临床分析

目的:总结白塞综合征的临床特点,提高对白塞综合征的认识.方法:对收集的128例白塞综合征患者的临床资料进行回顾性分析,对其临床表现、实验室检查、男女间的差异进行比较.结果:128例患者中男女比例约1.5:1,发病年龄多为20～39岁(78.3%).平均发病年龄(32.0±12.1)岁,病程1～22 年,平均病程5 年.其中不完全型80例(62.5%),完全型48例(37.5%).口腔溃疡是最常见的临床表现(128 例,100%),其次为皮肤损害(115 例,89.8%),生殖器溃疡(92 例,71.9%),针刺反应阳性(70例,54.7%),眼部损害(38 例,29.7%).人白细胞抗原(HLA)-B51 阳性率为29.5%(18/61).口腔溃疡为最常见的首发表现(91例,71.1%),其次为皮肤损害(69 例,53.9%),外阴溃疡(61 例,30.9%),眼部损害(15例,11.7%).男女间在发病率、皮损类型、眼部损害、神经系统损害及相关实验室检查差异显著.结论:白塞综合征累及多系统,临床表现多变,男女间差异明显.     

甲下鲍恩病6例临床及组织病理分析

目的：探讨甲下鲍恩病的临床、组织病理特征及鉴别诊断。方法：对2012年5月—2021年11月该院确诊的6例甲下鲍恩病患者的临床及组织病理资料进行回顾性分析。结果：所有患者均为男性，就诊年龄为45～77岁，病程为4个月～8年。6例患者肿瘤均位于手指甲，其中3例表现为纵行黑甲，1例表现为甲下出血，1例表现为甲板增厚、变形，局部呈疣状外观，1例表现为甲下角化过度。所有患者甲组织病理均表现为典型鲍恩病改变。结论：甲下鲍恩病可表现为纵行黑甲。该病临床容易误诊，及时行组织病理检查有助于明确诊断。     

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。     

秋水仙碱治疗Behcet氏病的皮肤症状

作者用秋水仙碱治疗5例 Behcet 氏病。患者33～59岁,男性3人,女性2人。有结节红斑样损害,口腔溃疡、阴部糜烂和毛囊炎样皮疹,多数有眼部症状,病程1～8年,曾用非皮质类固醇抗炎药或并用皮质类固醇治疗效果不满意。后用秋水仙碱治疗,每日口服1mg,不到一个月,皮肤和眼症状均明显好转,血沉和 C 反应球蛋白(CRP)也恢复正常。在每日1mg 秋水仙碱用药两个月到两年间,5例患者均取得了明显效果,能从事正常活动,症状无复发,     

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…     

Behcet病皮肤损害的观察

Behcet病(简称BD)是一慢性进行性复发性多系统损害疾病,皮肤损害是常见损害之一.我们对1972-1990年所见的符合1987年日本BD研究委员会诊断标准[最新医学,1988,43: 382]的312例患者的皮肤损害进行了研究,并对其在本病中的地位和诊断价值作一探讨.     

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。     

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.     

Hidradenitis suppurativa of the nape: Description of an atypical phenotype related to severe early‐onset disease in men

Some patients with hidradenitis suppurativa (HS) develop severe inflammatory lesions of the nape. Through a single‐center, cross‐sectional study with a total of 377 patients, we sought to compare patients with and without nape involvement, to determine whether disease severity is greater in these patients and to describe their clinical characteristics. Thirty patients (90% male) were identified as having nape involvement. Patients with versus without nape involvement had greater disease severity, earlier disease onset, a family history of HS, lower body mass, and higher Dermatological Life Quality Index and pain scores. Amongst them, involvement of the trunk and gluteal regions predominated. A significant univariate relationship existed between patients with nape involvement and male sex, early disease onset, family history, scalp and gluteal involvement, Canoui‐Poitrine phenotypes II and III, and Dowling–Degos disease. A significant multivariate relationship existed with early disease onset, Dowling–Degos disease, and phenotypes II and III.     

Behçet''s Disease

Beh?et's disease is a multisystem disease featuring mucocutaneous, ocular, intestinal, articular, vascular, urogenital, and neurologic involvement. While classically intermittent in its manifestations, the disease can stabilize and become chronic in a given organ system. The diagnosis of Beh?et's disease is based on clinical criteria. Recurrent aphthous ulcerations in the mouth, skin lesions, eye lesions, and genital ulcerations must be present during the course of the disease for a diagnosis of Beh?et's disease to be made unequivocally. A nonspecific skin hyperreactivity called pathergy is said to be helpful in the diagnosis. There are no pathognomonic laboratory findings, but biopsy usually shows a venulitis. The pathogenesis of the disease is unknown. No virus has been satisfactorily isolated to date. There is evidence of an increased frequency of HLA-B5 and HLA-B12. Humoral and cellular immunity seem to play a major part in the pathogenesis of the various manifestations of the disease. The treatment of Beh?et's disease is difficult to evaluate because of the many spontaneous exacerbations and remissions during the clinical course of the disease. Topical corticosteroids for orogenital ulcers and ocular inflammation are helpful. Intralesional injections for affected joints and retrobulbar tissues are useful in some cases. Systemic corticosteroids have appeared to be helpful for all manifestations of the disease. It is customary to use 60 mg of prednisone by mouth daily during acute exacerbations, then to taper as the condition improves. Chlorambucil has been shown to be safe and effective for various manifestations of Beh?et's disease; often it is used in combination with corticosteroids.(ABSTRACT TRUNCATED AT 250 WORDS)     

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.     

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.     

Facial lichen striatus: successful treatment with tacrolimus ointment

We report a 22-year-old Japanese woman with facial lichen striatus (LS). The distribution of the lesions corresponded to that of Blaschko's lines. Histology of the lesional skin showed an inflammatory cell infiltrate around hair follicles and eccrine glands. Treatment of the linear lesions with tacrolimus ointment once or twice daily resulted in a dramatical improvement in a short time. LS is a T-cell-mediated inflammatory disease and tacrolimus ointment may be an effective alternative treatment for this disease especially when the lesions are located on the face.     

58例白塞病回顾性分析

目的：总结和分析白塞病的临床表现及诊治经验。方法：回顾性分析58例白塞病患者。结果：本组白塞病以女性、慢性型多见，好发于青壮年。21例急性型发病诱因以上呼吸道感染为主（15例，占71．43％），应注意病毒或细菌感染在急性型发病机制中的作用。临床表现以口腔、皮肤、生殖器、眼和关节受累常见。结论：针刺反应对本病特异性高。皮肤外伤后创面延迟愈合（23例，占39．66％）对诊断有重要价值。     

Necrotizing dermatitis due to Vibrio Vulnificus]

BACKGROUND: Vibrio vulnificus is a Gram-negative halophilic pathogen for man that can cause septicemia in patients with chronic liver disease. In healthy subjects, infections are generally local or regional. We report two cases of necrotizing dermo-hypodermitis due to Vibrio vulnificus. CASE REPORTS: The first patient was a 21-year-old fisherman who developed extensive necrotizing dermo-hypodermitis 24 hours after a penetrating scratch with a fish bone. The second patient was also a fisherman. This 35-year-old man developed a massive area of necrotic tissue on the left leg, also 48 hours after a penetrating fish bone scratch. Both patients exhibited neutrophil hyperleukocytosis and thrombocytopenia. Bacteriological study of the pus and damaged tissue identified Vibrio vulnificus. Blood cultures were negative. Liver tests and liver ultrasound were normal. The course was favorable in both cases with antibiotic treatment and excision of the necrotic tissue. DISCUSSION: For these two cases of Vibrio vulnificus necrotizing dermo-hypodermitis, the diagnosis was based on epidemiological, clinical and bacteriological arguments. Neither of these patients had liver disease, explaining the limited locoregional nature of the infection. The severity of the skin necrosis and its rapid course are related to the toxins produced by V. vulnificus. Curative medical and surgical care is generally effective in cases without septicemia and prognosis is good. Education of exposed subjects, fishermen and patients with chronic liver disease, should be helpful for prevention.