牛痘样水疱病样皮肤淋巴瘤与慢性活动性EB病毒感染的关系

目的 报道6例牛痘样水疱病样皮肤淋巴瘤,并研究其与慢性活动性EB病毒感染的关系.方法 临床病理分析、皮损免疫组织化学染色、血清学分析、EB病毒编码RNA原位杂交、外周血EB病毒DNA测定.结果 6例患者皮损均为反复发作的丘疹、丘疱疹、坏死、痘疮样瘢痕,其中4例还伴有程度不同的颜面、手足水肿.所有患儿均有长期间断发热等症状.皮损病理可见表皮多房性水疱,真皮全层大量淋巴细胞浸润,细胞形态异形,可见病理分裂象.4例皮损病理免疫组化染色,可见大量CD56阳性细胞,散在的CD3和CD45RO阳性细胞,T细胞内抗原-1和粒酶B染色阳性,诊断为牛痘样水疱病样皮肤NK/T细胞淋巴瘤;2例组化染色CD3和CD45RO阳性,CD56阴性,诊断为牛痘样水疱病样皮肤T细胞淋巴瘤.6例皮损均可见EB病毒编码RNA原位杂交阳性肿瘤细胞,血清学检查EB病毒衣壳抗原IgG抗体滴度升高,其中2例滴度为1:5120,2例为1:2560,2例为1:1280;2例患者外周血EB病毒DNA拷贝数高于正常.6例患儿均证实患有慢性活动性EB病毒感染.结论 牛痘样水疱病样皮肤淋巴瘤主要表现为颜面手足肿胀、水疱、痘疮样瘢痕,病理表现主要为真皮异形淋巴细胞浸润和血管中心坏死,免疫表型以NK/T型多见.慢性活动性EB病毒感染与该型淋巴瘤发病密切相关.     

种痘样水疱病样皮肤T细胞淋巴瘤1例

种痘样水疱病样皮肤T细胞淋巴瘤是以皮肤水肿性红斑、水疱破溃遗留凹陷性瘢痕,同时伴有发热和肝脾肿大的一种罕见的皮肤T细胞淋巴瘤.该病多见于儿童,与Epstein-Barr病毒(EBV)感染相关.1现将我们诊断的1例,报道如下.     

种痘样水疱病样淋巴组织增生性疾病研究进展

种痘样水疱病样淋巴组织增生性疾病是一种特殊的皮肤淋巴细胞增殖性疾病,与EB病毒感染有直接的关系。该病在东亚地区,包括日本、中国等地报告较多。种痘样水疱病样淋巴瘤容易发生在儿童,表现为面部、手背等光暴露部位为主的丘疱疹,愈后常遗留萎缩性瘢痕。患者常并发有系统表现,如发热、淋巴结增大,肝脾增大等,部分患者预后不良。种痘样水疱病样淋巴组织增生性疾病属于谱系性疾病,二者之间常无法严格区分,临床常见到种痘样水疱病转化为种痘样水疱病样淋巴瘤的病例。该文简要综述了种痘样水疱病样淋巴组织增生性疾病的临床和组织病理特点、发病机制以及治疗进展。     

种痘水疱样皮肤T细胞淋巴瘤1例

种痘水疱样皮肤T细胞淋巴瘤( hydro vaceiniforme-like cutaneous T- cell lymphoma,HVLCTCL)是一种与EB病毒慢性活动性感染相关的罕见的T细胞淋巴瘤,复习国内文献,自2006年渠涛等1报道以来,迄今报道不过20例.由于2005年WHO/EORTC皮肤淋巴瘤分类中,本病被认为是结外NK/T细胞淋巴瘤(鼻型)的一个特型,2而未具体命名,故迄今国外仍有多种名称,如原发性皮肤EB病毒相关性T细胞淋巴增生性疾病(primary cutaneous Epstein - Barr virus - assci—ated T - cell lymphoproliferative disordes)、种痘水疱样淋巴瘤(hydro vacciniforme- like lymphoma)3、EBV相关性皮肤NK/T -细胞淋巴瘤(EBV - associated cutaneous NK/T- cell lymphoma)4、水疱样淋巴瘤( hydro - like lymphoma)5、种痘水疱样原发性皮肤CD8阳性T细胞淋巴瘤(hydro vacciniforme - like primary cutaneous CD8 - positive T- cell lynphoma)6、种痘样皮肤T细胞淋巴瘤7等.相广才等则习惯用牛痘样水疱病样皮肤T细胞淋巴瘤.8现将我们所见1例报道如下,并对迄今为止国内相关皮肤科文献报道的18例患者的临床组织病理、免疫组化及EB病毒检测等情况结合近期国外文献进行了复习.     

非典型种痘样水疱病

报告1例非典型种痘样水疱病.患儿女,6岁8个月.反复发热、皮肤出现水疱4年,水疱愈合后遗留凹陷性瘢痕.皮肤科检查:面部、颈项部、臀部和四肢伸侧散在分布黄豆大凹陷性瘢痕,下肢可见数个暗紫红色浸润性斑块,压痛不明显,未见明显水疱.血清病毒学检测:EB(Epstein-Barr)病毒IgG抗体(EBV-cA-IgG Ab)(+).3年前曾行皮损组织病理检查:表皮水肿,水疱形成,真皮及皮下可见大量淋巴样细胞、中性粒细胞及少量嗜酸性粒细胞浸润,可见血管炎改变.皮损组织EBER原位杂交(+).诊断:非典型种痘样水疱病.     

伴面部严重肿胀的种痘样水疱病样皮肤T细胞淋巴瘤1例

报告1例伴面部严重肿胀的种痘样水疱病样皮肤T细胞淋巴瘤。患者男,23岁,面、颈、上胸部、双手背出现丘疹、丘疱疹、坏死、结痂、痘疮样瘢痕形成伴面部肿胀6年,加重4年。皮损组织病理及免疫组化检查符合种痘样水疱病样T细胞淋巴瘤。     

种痘水疱病样皮肤T细胞淋巴瘤

报告1例长期复发的种痘水疱病样皮肤T细胞淋巴瘤.患者女,47岁.从2岁开始反复发作水肿性红斑、水疱、坏死及痘疮样萎缩性瘢痕形成,冬轻夏重,主要累及面部和四肢.组织病理示真皮层内可见较多淋巴样细胞灶状和散在浸润,可见核分裂象.免疫组化检测显示淋巴样细胞CD2、CD3、CD4、CD5阳性,CD7、CD8少数细胞阳性,CD2...     

种痘水疱病样皮肤T细胞淋巴瘤

种痘水疱病样淋巴瘤是近年来认识到的一种皮肤T细胞淋巴瘤,儿童多见,主要累及面部和四肢,表现为水肿性红斑、水疱、结痂和坏死,可伴有高热、肝脾大等全身症状。与EB(Epstein-Barr)病毒感染有关。组织病理显示肿瘤细胞侵犯血管和脂膜。该文报告1例该病患者。     

种痘样水疱病样T淋巴细胞增生性疾病

目的:探讨种痘样水疱病样T淋巴细胞增生病的诊断和治疗方法,提高对该病的认识.方法:回顾分析1例种痘样水疱病样T淋巴细胞增生性疾病患者的临床表现、辅助检查、诊疗过程及预后等临床资料,并对相关文献进行复习.结果:18岁男性患者,2年来反复出现红斑、水疱、结痂和萎缩性瘢痕等症状,皮损分布于日光暴露部位,并伴有发热、肝脾大等全身症状.皮损组织学检查显示:真皮附件周围有大量淋巴细胞浸润,淋巴细胞成团聚集,偶可见淋巴细胞的细胞核分裂变形.免疫组化检查:皮损部位CD3、CD45RO和EB病毒IgM抗体反应为阳性,而CD20、CD79a、CD56、CD30、Ki-67、TIA-1抗体反应为阴性.该患者通过糖皮质激素和免疫调节药物治疗后,病情明显缓解.结论:种痘样水疱病样T淋巴细胞增生性疾病临床罕见,与EB病毒感染密切相关,如患者日光暴露部位的皮肤反复出现丘疹、水疱、坏死,并伴有发热,应高度怀疑该病可能性;糖皮质激素联合免疫调节药物可控制该病的症状,但应进行长期随访.     

湿疹样表现的淋巴瘤样丘疹病

报告1例湿疹样表现的淋巴瘤样丘疹病.患者男,57岁.躯干四肢散发红斑丘疹,渗出伴瘙痒1个月就诊.体检可见躯干及四肢散发绿豆至花生大小不一的红斑、丘疹,中央可见坏死,部分红斑表面渗出结痂,融合成斑块.皮损组织病理提示部分表皮坏死,周围棘细胞间水肿,形成水疱,真皮浅、中层小血管周围大片状淋巴、组织细胞浸润伴较多嗜酸性粒细胞,部分淋巴细胞核大有异形,可见到较多核分裂象.免疫组化染色结果示CD30(+),诊断为湿疹样表现的淋巴瘤样丘疹病.     

环氧化酶-2在表皮肿瘤中的表达

目的 探讨环氧化酶-2在不同表皮肿瘤中的表达及意义。方法 选择鳞状细胞癌8例、基底细胞上皮瘤10例、Bowen病8例和脂溢性角化病12例,运用免疫组化方法观察肿瘤细胞中环氧化酶-2的表达。结果 与正常表皮相比,环氧化酶-2在鳞状细胞癌、Bowen病、基底细胞上皮瘤中的表达明显上调,尤其以鳞状细胞癌中的表达最强。而环氧化酶-2在脂溢性角化病中的表达与正常人皮肤的表达近似。结论 环氧化酶-2表达的上调可能在表皮肿瘤的发生发展中发挥一定作用。     

Leprosy: a review of laboratory and therapeutic aspects - Part 2

Leprosy is a chronic infectious condition caused by Mycobacterium leprae(M. leprae). It is endemic in many regions of the world and a public health problem in Brazil. Additionally, it presents a wide spectrum of clinical manifestations, which are dependent on the interaction between M. leprae and host, and are related to the degree of immunity to the bacillus. The diagnosis of this disease is a clinical one. However, in some situations laboratory exams are necessary to confirm the diagnosis of leprosy or classify its clinical form. This article aims to update dermatologists on leprosy, through a review of complementary laboratory techniques that can be employed for the diagnosis of leprosy, including Mitsuda intradermal reaction, skin smear microscopy, histopathology, serology, immunohistochemistry, polymerase chain reaction, imaging tests, electromyography, and blood tests. It also aims to explain standard multidrug therapy regimens, the treatment of reactions and resistant cases, immunotherapy with bacillus Calmette-Guérin (BCG) vaccine and chemoprophylaxis.     

缓和性血清阴性对称性滑膜炎伴凹陷性水肿综合征一例并文献复习

目的了解缓和性血清阴性对称性滑膜炎伴凹陷性水肿综合征的临床表现。方法分析1例缓和性血清阴性对称性滑膜炎伴凹陷性水肿综合征患者临床表现、实验室检查及治疗反应。结果患者男,54岁,表现为对称性多关节炎及肢端水肿。血清类风湿因子、抗核抗体、抗ds—DNA抗体、抗合成循环瓜氨酸多肽抗体、免疫球蛋白、补体均正常。x线检查示双手掌指骨骨质破坏。糖皮质激素和免疫抑制剂治疗效果好。结论缓和性血清阴性对称性滑膜炎伴凹陷性水肿综合征是一组异质性疾病。     

白塞综合征患者抗血管内皮细胞抗体和抗心磷脂抗体的检测

目的 探讨血清抗血管内皮细胞抗体(AECA)及抗心磷脂抗体(ACA)在白塞综合征发病中的作用。方法 收集白塞综合征患者和正常人血清,用间接免疫荧光技术检测血清中AECA并分型,用酶联免疫吸附方法检测ACA。结果 白塞综合征组AECA阳性率为81.3%(32例中26例阳性),其中IgG-AECA阳性率为71.9%,IgM-AECA阳性率为53.1%,与正常人对照组比较差异有显著性(P<0.01)。同时AECA阳性组与阴性组比较在发生结节性红斑或结节性血管炎及血沉增快方面前者高于后者。ACA检测结果,白塞综合征组阳性率为53.1%,显著高于正常人对照组。ACA阳性组与阴性组比较,在口腔溃疡、皮肤、外生殖器及贫血等临床表现及血沉等方面差异无显著性(P>0.05)。结论 白塞综合征患者血清AECA水平对白塞综合征血管损害及病情活动具有一定意义。ACA对病情的影响尚需进一步研究。     

线状和回旋状色素沉着症并发炎性线状表皮痣及寻常性鱼鳞病

报告1例线状和回旋状色素沉着症并发炎性线状表皮痣、寻常性鱼鳞病。患者女,17岁。2岁起胸背部出现线状和回旋状色素沉着,双下肢皮损覆鱼鳞状鳞屑,双手背关节和左侧膝部、踝部、足背关节处可见线状鳞屑性红斑。系统及实验室检查均正常。结合组织病理改变诊断为线状和回旋状色素沉着症并发炎性线状表皮痣、寻常性鱼鳞病。     

Update summarising the conclusions of the international consultation on male lower urinary tract symptoms

The International Consultation on Urological Disease have recently published comprehensive conclusions, based on evidence reviewed by eight committees, on aspects of male lower urinary tract symptoms(LUTS). In this review, we summarise the conclusions from fourof the committees, namely, the evidence regarding the epidemiology of male LUTS, patient assessment, nocturia and medical management. It is indisputable that with an expanding and ageing global population the prevalence of male LUTS is likely to increase. Therefore symptom prevention and preservation of quality of life(Qo L) feature highly in the guidelines. There are now a number of different medical options, proven to lead to significant improvements in symptom scores, flow rate and Qo L available to men with LUTS. Metaanalyses have shown the benefits for alpha blockers, antimuscarinics, 5-α reductase and phosphodiesterase-5 inhibitors. High level evidence also exists for combinations of all of the above with alpha blockers and so men with concomitant storage symptoms, prostate volume 30 mL, PSA 1.4 or erectile dysfunction may be considered for combination treatment of an alpha blocker with an antimuscarinic, 5-α reductase inhibitor or phosphodiesterase-5 inhibitor respectively. In an era of personalised medicine, appropriate patient selection is likely to provide the key to the most effective clinical management strategy.     

Sweet side of bladder cancer

The malignant transformation of cells is often accompanied by deranged expression of the sugar chains,i.e.,glycans,attached the cancer cell surfaces or attached to secreted proteins.The aberrant expression of specific glycans in bladder cancer has also been reported by several research groups.Similarly to other cancers,glycans such as the sialyl Tn antigens have been suggested as diagnostic and prognostic biomarkers of bladder cancer,and associated with disease progression and patient’s response to treatment.At present our understandings about the role of glycans in bladder cancer is still limited,but at the same time it is now assumed that this understanding urges and it will fuel the development of novel strategies of diagnostic and therapy.