国内这些年来有越来越多的人利用网络信息技术开始实施各种各样的犯罪活动,最突出的就是在网上出现很好诽谤造谣的现象,把公民的基本权益给伤害了,把社会的秩序给扰乱了,更严的是还发生群体件,严重影响社会的和谐与稳定.所以我国的最高级人民检察院与人民法院一起颁发了《有关利用办理网络信息的诽谤案件的若干法律问题解释》(下文称为《解释》),并已经开始施行.笔者根据《解释》及违法犯罪现象,梳理了相关的司法解释,进一步确定了网络诽谤等违法行为的定罪量刑标准.

Objective To evaluate the protective effect of polysaccharide nucleic acid fraction of bacillus Calmette-Guerin (BCG-PSN) against atopic dermatitis (AD) in Nc/Nga mice,and to explore its possible mechanism.Methods Sixteen Nc/Nga mice were classified into normal control group (n =4),low-concentration BCG-PSN group (n=5) and high-concentration BCG-PSN group (n =7) to be subcutaneously injected with sodium chloride physiological solution,BCG-PSN of 0.1 mg/kg and 0.5 mg/kg respectively,at 1,8,15 and 22 days of age.Dinitrochlorobenzene (DNCB) was repeatedly and topically applied to these Nc/Nga mice to induce AD-like lesions at 49 days of age.The preventive effect of BCG-PSN against AD was evaluated by dermatitis scores,scratching frequency,histopathological manifestations and immunological parameters (including IgE,i nterleukin (IL)-4 and-12,and interferon (IFN)-γ).Results Repeated injection of BCG-PSN within 4 weeks after birth significantly decreased the severity of DNCB-induced AD-like lesions,dermatitis scores and scratching behavior in Nc/Nga mice.There was no statistical difference in scratching frequency between the high-and low-concentration BCG-PSN groups.BCG-PSN treatment reduced the plasma level of IgE in Nc/Nga mice in a dose-dependent manner.BCG-PSN at 0.5 mg/kg increased the number of cells secreting IFN-γ in skin lesions of mice.Both doses of BCG-PSN down-regulated IL-4 level,but up-regulated IL-12 level in the culture supernatant of spleen mononuclear cells from mice.Conclusion Early injection of BCG-PSN could protect Nc/Nga mice against dermatitis by promoting the proliferation of IFN-γ-secreting cells,increasing the synthesis of IL-12,and reducing the levels of IL-4 and IgE.

人型支原体(Mycoplasma hominis,Mh)是引起非淋菌性尿道炎常见的病原菌之一,近年来由于广谱抗生素的广泛应用、反复感染及慢性迁延等原因,导致Mh临床分离株耐药性日益严重,抗生素治疗效果下降.1为了解本地区Mh感染的耐药趋势,对我院皮肤性病门诊近6年来分离到的Mh进行了药物敏感性试验,以期总结本地区Mh耐药情况的变化趋势.

解脲脲原体(Ureaplasma urealyticum,Uu)是引起非淋菌性尿道炎最常见的病原菌之一,近年来Uu 临床分离株耐药性日益严重,抗菌药物治疗效果下降.为了解本地区Uu感染的耐药趋势,对我院皮肤性病门诊2003-2008年分离到的Uu进行药物敏感试验,以期总结本地区Uu耐药规律的变化趋势.

目的: 研究一Hartnup病家系的氨基酸转运蛋白基因(SLC6A19)的突变.方法: 提取Hartnup病患者及家族成员的基因组DNA,采用聚合酶链反应(PCR)扩增SLC6A19基因所有的外显子,并对PCR产物进行测序序列分析.结果: Hartnup病患者SLC6A19基因存在异常:第6外显子第850位碱基由鸟嘌呤变为腺嘌呤,使第284位氨基酸由甘氨酸(G)转变为精氨酸(R),即G284R错义突变.其弟与患者突变相同.其父母均为G284R突变杂合子.结论: 该Hartnup病家系由氨基酸转运蛋白基因(SLC6A19)的G284R错义突变所致.

原发性红斑肢痛症是一种少见的先天性常染色体显性遗传病.致病基因于2003年首次被定位于2号染色体2q24.3的SCN9A基因[1].我们在临床工作巾收集到两个原发性红斑肢痛症家系,并对其致病基因进行研究。

A case of Papillon-Lefevre syndrome is reported. The patient was a 23-year-old male, who developed gingivitis within the first month of life and presented with plantar keratoderma 4 years ago. He also had fissured tongue when he was 3 years old. The lesions got improved by administration of acitretin.

临床资料患者,24岁.因躯干、四肢皮疹3年,上胸部皮疹2年,于2004年3月就诊于我科门诊.患者就诊前3年无明显诱因于颈部出现大片棕褐色扁平皮疹,无明显不适.

患者女,41岁.因左面部肿胀半年,于2002年11月入我院治疗.半年前患者无明显诱因于左上眼睑内侧出现一小指甲盖大红斑,伴肿胀,1周后肿胀蔓延至左面上半部及右眼眶周,自觉左眼眶上部轻度疼痛,皮损经日晒后加重.曾在外院诊断为慢性丹毒,给予青霉素静脉滴注,无明显疗效.自发病以来,睡眠较差,两便正常.否认面部有外伤史.

Objective to discuss all of factors which affect the result of dermatologic surgery by the way of fishbone. Methods Fishbone is common useful, intuitionistic and logistic method of management. It is benefit to analyse the causality. Results fishbone shows that there are forties factors affecting the results of dermatologic surgery. Conclusion dermatologic surgery is a systems engineering. Technique of operation is essential but inadequate. Being a dermatologist, we must control all the factors which were showed in fishbone in order to get the success of our operation.

患者女,23岁.颈部、腋下反复起脓疱10年,泛发全身伴发热2个月,于2004年5月入院治疗.患者10年前无明显诱因颈部、腋下出现许多小脓疱,经治疗可消退,但反复发作,每年发作约10余次.皮损局限于颈部、腋下,无发热.5个月前怀孕,2个月前皮损突然增多,泛发全身,伴有发热,体温最高达39.5℃.家庭成员中无类似疾病患者.皮肤科检查:面颈部、躯干大片红斑,表面覆黄色痂皮,脱屑,背部许多粟粒大脓疱,部分脓疱表面结痂.双耳后、发际处及项背部见多处红斑,上覆细小鳞屑(图1A、B);双手掌、手背多处暗红色圆形红斑,中心颜色较深,边缘颜色较浅.20甲正常,口腔、外阴黏膜正常.实验室检查:血红蛋白87g/L,生化全项及尿、粪常规均正常,血沉62 mm/1 h,血钙正常,白蛋白28.2 g/L.多次取脓疱液培养均无细菌生长.皮损组织病理学检查:棘层肥厚,角层下脓疱,中性粒细胞海绵水肿;真皮浅层有中性粒细胞、嗜酸性粒细胞浸润(图2).间接免疫荧光检查阴性.诊断:疱疹样脓疱病.入院当天给予地塞米松5 mg肌内注射后,体温降至37.5℃以下,第3天因体温上升并出现新发皮损,遂给予泼尼松30 mg/d口服,3周后因体温再次升高,将泼尼松口服剂量增至60mg/d,至妊娠9个月时,剖宫产下一健康男婴.分娩后给予阿维A 30 mg/d口服,治疗近2周体温逐渐恢复正常,皮损也逐渐消退.

Objective To identify C0L7Al gene mutations in a family of recessive dystrophic epidermolysis bullosa (RDEB). Methods PCR and direct DNA sequencing were used to determine the mutation sites and types. PCR using allele-specific oligonucleotide primers was performed to further identify the pathogenic cause of this disease. Results The patient examined in this study was a compound heterozygote for a S48P missense mutation in exon 2 and a 3625del 11 PTC mutation in exon 27, which was a novel combination of COL7Al mutations in RDEB. Conclusion The missense mutation and the nonsense mutation in COL7Al gene are underlying causes of the Hallopeau-Siemens variant of RDEB.

Here is reported a case of Behcet’s disease mimicking psoriasis pustulosa in a 59-year-old woman. She pre-sented with generalized lesions all over her body including papules and pustules with itching lasting for over 3 months, dis-seminated maculopapules on her trunk and upper limbs more than 1 month, recurrent oral ulcer during the last 3 years and iridocyclitis for 2 years. After hospitalization grouped pustules appeared all over her body with hyperpyrexia at night. The histopathology showed a leukocytoclastic vasculitis. Therapy with prednisone worked effectively.

Objective: To detect gene mutations in a Chinese family with primary erythermalgia. Methods: Mutations in SCN9A gene were detected by PCR amplification of the 26 coding exons of SCN9A and sequencing of the PCR products, and compared the sequences in this family with the sequence from gene database. Results: A new mutation was found in this pedigree, and the cytosine in the base number 2572 of this gene was replaced by thymine with the corresponding amino acid leucine replaced by phenylalanine. Conclusions: A new mutation is detected in this family, and new evidence has been found to prove the SCN9A is the responsible gene for primary erythermalgia. Also, this finding is helpful to investigate the mechanism of this disease and provide evidence to treat it.

OBJECTIVE:To identify the DSRAD gene; mutations in three Chinese families with dyschromatosis symmetrica hereditaria.METHODS:All exons of DSRAD gene were analyzed in each person of these families with PCR-DNA sequencing. DNA samples from 100 unrelated, normally pigmented adult individuals were also included as control.RESULTS:We identified a missense mutation of C3220T (R1074C) in DSRAD gene in family A, and another missense mutation of G3325T (D1109Y) in DSRAD gene in family B and C. No same mutation was found in unaffected individuals in the families and the controls.CONCLUSION:We found two special missense mutations in DSRAD gene in three families of dyschromatosis symmetrica hereditaria. These mutations may impair DSRAD protein function, and as a consequence, cause skin dyschromatosis.

1病例资料 病史:患者女,22岁.因全身皮疹9个月于2002年12月收入院.9个月前患者口腔、外阴部出现疼痛性溃疡,8个月前双手指端出现许多针尖大红色皮疹.7个月前患者四肢出现大小不等的水疱,疱壁易破,且逐渐累及全身.双手掌、手背皮肤出现大面积糜烂、溃疡,渗出较多,表面有较厚的结痂.在外院曾被诊断为"白塞病"、"天疱疮"等,应用大剂量糖皮质激素治疗效果不明显.近1个月来患者出现发热、胸闷,遂从当地转来我院.患者既往体健,否认有药物过敏史.

目的 对原发性红斑性肢痛症的致病基因进行定位及突变研究.方法收集一个原发性红斑性肢痛症家系成员和一个散发病例的血样抽提基因组DNA.选用2号染色体长臂上已知致病区域的6个微卫星标记对该家系成员进行基因扫描,并对基因分型结果进行连锁分析及单倍型分析.PCR扩增SCN9A基因的全部外显子,并进行测序.针对所发现的突变以Hph Ⅰ、BsrS Ⅰ内切酶行限制性片段长度多态性(RFLP)分析.结果连锁分析结果发现本家系在微卫星标记D2S2370和D2S2330的两点最大LOD值为2.11(重组率=0.00),单倍型分析发现本家系在微卫星标记D2S1353和D2S2345存在重组.家系患者和散发病例均存在SCN9A基因第15外显子的错义杂合点突变:家系全部患者均存在T2573A杂合突变,散发病例存在T2543C杂合突变,分别导致Nav1.7第858位的亮氨酸被替换为组氨酸(L858H)及第848位的异亮氨酸被苏氨酸替换(I848T).RFLP证实了正常对照无此突变.结论在世界上首次证明SCN9A基因是原发性红斑性肢痛症的候选致病基因。

患者女,48岁,因躯干红斑、疱疹伴痒6个月,于1986年4月8日收入院.6个月前患者双腋下、腰部出现红斑、水疱、脓疱,痒,1周自然破溃结痂,并逐渐发展到胸背、大腿,有些皮疹环状排列,分批出现,原因不明,与饮食无关.外院按湿疹皮炎治疗,疗效不佳,遂来我院诊治.发病以来,无其他不适.否认职业环境接触史.体检:一般情况好,各系统检查未见异常.双耳后、颈、双腋下、胸背中部、双腹股沟、臀可见红斑,其上及边缘处分布较多脓疱,粟粒至黄豆大,疱壁薄、松弛,部分融合成脓湖,Nikolsky征阳性.皮损面积占体表面积40%.外阴、掌跖、指甲无皮损.实验室检查:入院后进行血、尿、粪常规检查,无异常.脓疱内容物培养无菌生长,蛋白电泳:γ-球蛋白0.231,血钙2.15 mmol/L.皮损直接免疫荧光阴性.组织病理示角层下脓疱,内有多数中性粒细胞浸润.真皮上部血管周围炎症细胞浸润.诊断:角层下脓疱病.

患者男,56岁,因龟头、冠状沟、包皮内侧持续性红斑9年于2000年12月来我科就诊.患者9年前无明显诱因于龟头处出现红斑,持续不退,逐渐增大,后发展至冠状沟及包皮内侧,表面少许无色分泌物,无自觉症状.自行外用抗生素软膏无效.患者既往体健,无结核和其它传染病史,无长期服药史,否认婚外性接触史.