冷球蛋白血症

患者男,66岁,因双手皮肤出现紫红斑8年余,局部关节疼痛1年,于2003年1月8日就诊于我院皮肤科门诊,患者近8年来每年冬季双手皮肤出现紫红斑,皮温下降,症状持续至环境温度上升后才能恢复正常肤色,自觉有刺痛和麻木感,双腕及双膝关节间歇性疼痛1年余,无肿胀及治动受限,8个月前双下肢曾出现水肿,无腰痛,尿频、尿急,尿痛和血尿史。不规则饮酒20余年,1年前已戒酒,既往有“胃病”史,无输血史,家族成员中无类似疾病史。     

冷球蛋白血症的发病机理

冷球蛋白血症发病机理的研究是一个十分复杂而又相当有趣的课题。本文从免疫学说,冷作用,感染因素,遗传因素及其它等五个方面综述了冷球蛋白血症发病机理,对其进一步深入研究,将有助于阐明自身免疫性疾病和各种淋巴组织增生性疾病的本质。     

冷球蛋白血症的发病机理

冷球蛋白血症发病机理的研究是一个十分复杂而又相当有趣的课题。本文从免疫学说，冷作用，感染因素，遗传因素及其它等五个方面综述了冷球蛋白血症发病机理，对其进一步深入研究，将有助于阐明自身疾病和各种淋巴组织增生性疾病的本质。     

冷球蛋白血症

1933年Wintrobe等首先在1例多发性骨髓瘤患者血清中发现了一种在低温(4℃)发生沉淀,37℃左右又溶解的蛋白质,1947年Lerner等人命名为冷球蛋白,当血中冷球蛋白增高(>25mg/mL)可引起冷球蛋白血症。冷球蛋白血症是一种系统性血管炎,常继发于中小血管循环免疫复合物的沉积,通常表现为典型的三联征(紫癜、无力和关节痛)1和一个或一个以上的器官受累如慢性肝炎、肾小球肾炎、周围神经病变、皮肤溃疡及播散性血管炎。1分类和分型冷球蛋白血症分为原发性和继发性,以后者居多,约占60%～75%,在皮肤科领域后者存在于系统性红斑狼疮、结节性多动脉炎、…     

冷球蛋白血症性股臀皮肤血管炎1例

冷球蛋白血症性股臀皮肤血管炎为临床上少见的一种皮肤病，1994-2002年8月，医学核心期刊共收录4篇相关的文献报道，病例数累计25例。现将我们诊断的1例报道如下。     

丙型肝炎病毒感染的皮肤表现

现认为1989年发现的丙型肝炎病毒（HCV）是非经肠道传播性病毒性肝炎，是输血相关性肝炎的主要原因之一[1]。HCV可以感染静脉注射接受者（除输血外，静脉毒瘾者也是主要的）、器官移植受者、进行血液透析的病人以及医务人员等。其感染的特点是易慢性化，据统计，约50％的急性丙型肝炎感染将发展为慢性肝病，其中约20％将进展为肝硬化，并可能演变成肝细胞性肝癌[2]。HCV是一种单链RNA病毒，与登革热病毒和黄热病毒属同一族，经由胃肠道外的途径进入体内。慢性丙型肝炎最常见的临床表现为乏力，而黄疽少见；其实验室特征与乙型肝炎相似…     

冷球蛋白血症性股臂部皮肤血管炎1例

临床资料，患者，女，23岁，因双股外上方和臂部外侧出现红色皮疹，伴轻度瘙痒3个月来诊，患者于3个月前无明显诱因双股外上方和臂部外侧出现料粒大小红色丘疹，以后皮疹逐渐增大，增多，     

系统性红斑狼疮患者合并丙型肝炎病毒感染的临床分析

目的:确定系统性红斑狼疮(SLE)患者丙型肝炎病毒(HCV)感染的发病率及其临床意义。方法:用ELISA-3、RIBA-3及PCR检测134例SLE和200名正常献血者HCV感染情况。结果:15例SLE患者(11%)和2名对照组(1%)HCV抗体阳性(P<0.001),SLE患者合并HCV感染率明显高于对照组,合并HCV感染SLE患者的皮肤表现、ds-DNA抗体发生率低(P=0.01,P<0.001),肝脏损害、低补体血症、冷球蛋白血症发生率高(P<0.001,P=0.002,P=0.03,P=0.02)。结论:SLE患者的HCV感染率高于正常人群。     

伴冷球蛋白血症和丙型肝炎病毒感染的白细胞碎裂性血管炎

冷球蛋白血症是一种表现为血管炎的系统性疾病,特征为典型的临床三联征(紫癜、虚弱、关节痛),有时伴有慢性肝炎、外周神经病和肾小球肾炎。血管内常能观察到类风湿因子(RF)和循环免疫复合物沉积。曾提出多种病毒如乙型肝炎病毒、EB病毒常是混合性冷球蛋白血症的致病因子。该文报告1例伴有丙型肝炎病毒感染和双下肢可触及的紫癜的单克隆IgAκ-多克隆IgG混合性冷球蛋白血症。     

伴发丙型肝炎病毒感染的皮肤病住院患者34例临床分析及文献复习

丙型肝炎病毒(HCV)是一种可引起慢性病毒性肝炎的黄病毒科成员,除引起肝炎外,HCV还可累及网状淋巴系统、肾脏、皮肤等多个肝外器官.据报道,高达74％的丙肝患者至少有一种肝外器官受累表现,其中有过皮肤表现的约占17％[1].尤其是近年来,我国HCV感染率逐年上升.国外文献报道已证实与HCV感染直接相关的皮肤病包括混合性...     

Urticarial Vasculitis with Papular Lesions in a Patient with Type C Hepatitis and Cryoglobulinemia

We describe a case of urticarial vasculitis accompanied by erythematous wheals, palpable pupura, and subsequent necrotic ulcerated papular lesions in a patient with type C chronic hepatitis and type II cryoglobulinemia (IgM-kappa and polyclonal IgG). A 56-year-old man developed recurrent urticarial lesions on his lower extremities and trunk. The histology revealed leukocytoclastic vasculitis with perivascular immunoglobulin deposits. Subsequently, multiple reddish papular lesions with necrotic ulcerations appeared on the extensor aspect of his extremities and buttocks. Histology of these lesions showed cryoglobulinemic vasculitis with prominent fibrinoid necrosis of the vascular walls and cryoprecipitate within the vasculature as well as increased hyalinized collagen bundles. These papular lesions have not previously been described as cutaneous necrotizing venulitis to the best of our knowledge. It is suggested that the immune response to hepatitis C virus infection and cryoglobulins may be responsible for severe necrotizing venulitis, resulting in unusual cutaneous lesions.     

Hepatitis C: a professional risk for dermatosurgeons

Background Health care workers (HCW) in close contact with blood or serum are at risk for professional contagion with HCV. Among such personnel, the dermatosurgeon is on the firing line. We tried to quantitate such risk. Design One hundred consecutive out-patients seeking dermatosurgical advice for cutaneous neoplasms were studied for serologic markers for HCV infection. Results Only 34 patients had not contracted any of the known hepatitis viruses, and 8% of the patients were anti-HCV-positive. Conclusion On the basis of these findings and of data in the literature, we conclude that in Genoa HCW run a high risk of acquiring HCV infection due to needle stick injury1. Patients should undergo serologic investigation for HCV infection before surgery, even a minor one, is performed.     

人免疫缺陷病毒合并丙型肝炎病毒的调查及相关因素分析

目的对人类免疫缺陷病毒(human immunodificiency virus,HIV)感染者中合并丙型肝炎病毒(hepatitis C virus,HCV)感染的情况进行流行病学调查和统计学分析,评估合并感染的患病率和相关影响因素。方法对各医院在2006—2008年收治的门诊和住院HIV感染病例,用统一的流行病学调查表进行登记;并进行HCV抗体、肝功能和CD4细胞检测;多元回归法分析HIV合并HCV感染风险因素。结果 HIV感染者978例,合并HCV的感染率为33.9%,静脉吸毒途径感染HIV占合并感染者的81.3%,30~45岁年龄组HIV合并感染率为42.6%,无业人员HIV合并感染率为61.9%,均明显高于其他组。结论年龄30~45岁和无正当职业的人群HIV合并HCV感染率较高,静脉吸毒是HIV合并HCV感染的主要传播途径。     

Hepatitis C and renal transplantation in era of new antiviral agents

Data from World Health Organization estimates that the hepatitis C virus (HCV) prevalence is 3% and approximately 71 million persons are infected worldwide. HCV infection is particularly frequent among patients affected by renal diseases and among those in dialysis treatment. In addition to produce a higher rate of any cause of death, HCV in renal patients and in renal transplanted patients produce a deterioration of liver disease and is a recognized cause of transplant glomerulopathy, new onset diabetes mellitus and lymphoproliferative disorders. Treatment of HCV infection with interferon alpha and/or ribavirin had a poor efficacy. The treatment was toxic, expensive and with limited efficacy. In the post-transplant period was also cause of severe humoral rejection. In this review we have highlighted the new direct antiviral agents that have revolutionized the treatment of HCV both in the general population and in the renal patients. Patients on dialysis or with low glomerular filtration rate were particularly resistant to the old therapies, while the direct antiviral agents allowed achieving a sustained viral response in 90%-100% of patients with a short period of treatment. This fact to date allows HCV patients to enter the waiting list for transplantation easier than before. These new agents may be also used in renal transplant patients HCV-positive without relevant clinical risks and achieving a sustained viral response in almost all patients. New drug appears in the pipeline with increased profile of efficacy and safety. These drugs are now the object of several phases II, III clinical trials.     

Palpable purpura at the site of previous herpes zoster in association with mixed cryoglobulinemia and hepatitis C virus infection.

A 70-year-old woman affected with chronic active hepatitis C and mixed cryoglobulinemia presented a palpable purpura on her abdominal skin in a metameric configuration, fourteen months after a herpes zoster in the same site. Histopathology showed a small vessel leukocytoclastic vasculitis in the superficial dermis. Post-zoster eruptions are variable, and their spectrum is still expanding, although the pathogenesis remains to be elucidated. Perhaps our case represents an isomorphic reaction, because this palpable purpura, probably related to HCV infection, occurred several months after herpes zoster.     

Clinical and Histopathologic Analysis of the Relationship between Lichen Planus and Chronic Hepatitis C

A prospective clinical investigation of 45 patients with lichen planus (LP) demonstrated a significant association between LP and chronic hepatitis C. Anti-hepatitis C virus (HCV) antibodies were found in 17 (37.8%) of the 45 LP patients. This was significantly higher than in the controls. This higher prevalence of anti-HCV antibodies was found equally in both male and female patients in the three types of LP; cutaneous only type, mucous only type, and both cutaneous and mucous type. Most of the patients with positive anti-HCV antibodies had abnormal values of transaminase enzymes and/or a past history of chronic hepatitis. Histological and immunohistological investigations of three cases with LP and chronic hepatitis C demonstrated some morphologic similarities between these two diseases. Histopathologic findings of both LP and chronic hepatitis C were based on a T lymphocytic infiltrate with keratinocyte or hepatocyte damage. The degrees of infiltrating cells positive to UCHL-1, MX-panB, Leu-7, and human leukocyte antigen (HLA)-DR antibodies in the chronic hepatitis C lesions seemed to be similar to those in the LP lesions. These results may support a possible relationship between LP and chronic hepatitis C and the hypothesis that LP may be associated with chronic liver diseases as a result of a cytotoxic attack on the hepatocytes.     

Purpura with cold urticaria in a patient with hepatitis C virus infection-associated mixed cryoglobulinemia type III: successful treatment with interferon-beta

We describe a 54-year-old man with hepatitis C virus (HCV) infection-associated cryoglobulinemia type III. The patient had suffered from cold-induced urticaria that left purpuric eruptions up to 1 cm in diameter, intermittent migratory joint pain for seven years and mild liver dysfunction for nine years. Hemophilia A was diagnosed when the patient was 26 years old, and he was then given infusions of factor VIII for a short time. In both skin biopsy samples from urticarial and purpuric eruptions, mild inflammatory infiltration by polymorphonuclear leukocytes with nuclear dust, extravasation of erythrocytes and deposition of IgM and C3 in the superficial blood vessels were observed. After antiviral treatment with interferon-beta, the clinical symptoms and the cryoglobulin and HCV-RNA in the serum disappeared. There has been no recurrence in the subsequent nine years.     

玫瑰糠疹与人类疱疹病毒-7型关系的研究

目的探讨玫瑰糠疹(PR)与人类疱疹病毒-7型(HHV-7)的关系。方法采用巢式PCR检测了22例PR急性期患者的血浆、外周血单核细胞(PBMC)、皮损、唾液、尿液,14例恢复期患者的唾液、血浆、外周血单核细胞,14例正常人的唾液、血浆、外周血单核细胞中的HHV-7特异性序列。结果急性期唾液与单核细胞中HHV-7DNA检出率(95.5%,45.4%)明显高于正常人(64.3%,21.4%),唾液中HHV-7DNA检出率亦明显高于恢复期(57.1%),但单核细胞中HHV-7DNA检出率与恢复期(28.6%)无明显差异。8例皮损组织(36.4%)检测到HHV-7DNA。1例血浆中检测到HHV-7DNA(4.54%),其外周血单核细胞、皮损、唾液中亦均检测到HHV-7DNA,其恢复期唾液、单核细胞中HHV-7DNA仍可检测到。恢复期HHV-7DNA在唾液和单核细胞中的检出率与正常人无明显差别。结论有一部分玫瑰糠疹的发生可能与潜伏的HHV-7活化感染有关。     

Incidence and prevalence of hepatitis B and hepatitis C viruses in hemodialysis patients in Lebanon

AIM: To determine the incidence and the prevalence of hepatitis B and C viral infections in patients on hemodialysis (HD) across Lebanon.METHODS: We reviewed the data registry at the Lebanese Ministry of Public Health where records of monthly hepatitis B surface antigen (HBsAg) and hepatitis C virus (HCV) serology are reported from 60 affiliated HD centers across Lebanon. All patients who were on HD or who started HD between October 2010 and July 2012 were included in the study. Patients from seven HD centers were excluded due to inadequate and incomplete results reporting. During the selected period, HBsAg and HCV serology were available for 3769 patients from 53 HD centers distributed at all Lebanese governorates. The prevalence was calculated by dividing the number of patients with positive HBsAg or HCV serology to the total number of patients. The Incidence was calculated by dividing the number of newly acquired infection to number of patients-years (p-y). Incidence rates at different governorates were compared to each other using two tailed Z test and a P value of < 0.05 was considered significant.RESULTS: Sixty out of 3769 HD patients were found to have positive HBS Ag and 177 out of 3769 were positive for HCV Antibodies. The prevalence of hepatitis B virus (HBV) and HCV in HD patients across Lebanon was 1.6%, and 4.7%, respectively. The comparison of prevalence according to geographic distribution could not be done accurately due to the frequent shift of patients between dialysis centers at different governorates. The incidence rate was 0.27 per 100 p-y for HBV and 0.37 per 100 p-y for HCV. There was no significant difference concerning the incidence of HBV between HD centers at different governorates (all P values > 0.1), but this difference was highly significant concerning the incidence rates of HCV which occurred predominantly in the southern centers (1.47 per 100 p-y) with a P value of 0.00068 and 0.00374 when compared to Mount Lebanon (0.21 per 100 p-y) and the Northern centers (0.19 per 100 p-y), respectively.CONCLUSION: The incidence rate of HBV and HCV is very low in the Lebanese HD centers and their prevalence is decreasing over the last two decades.