原发干燥综合征45例临床分析

目的:分析原发性干燥综合征(pSS)的临床特征,旨在提高对该病的诊治水平.方法:回顾性分析该院住院诊治的45例pSS患者的临床特点.结果:成人发病者首发症状表现多样化,常见的有口眼干燥、关节疼痛、淋巴结肿大、血小板减少、腮腺肿大、发热及皮肤表现等.结论:pSS的首发症状多样化,易误诊、漏诊,仔细询问病史并进行自身抗体等相关检查是避免误诊的关键.     

原发性干燥综合征患者伴发中枢神经病变的相关风险因子分析

目的:探讨原发性干燥综合征伴发中枢神经病变(pSS-CNS)的临床特点及危险因素.方法:将2015年1月至2020年10月南方医科大学南方医院确诊的291例原发性干燥综合征(pSS)患者,分为pSS-nCNS组(246例)和pSS-CNS组(45例),收集两组患者的一般情况、临床表现、实验室检查结果等临床资料,分析两组...     

干燥综合征伴静脉血栓3例

1临床资料 3例原发性干燥综合征患者诊断符合2002年修订的干燥综合征国际诊断标准。2例因下肢静脉血栓、下肢肿胀首诊人院,后确诊为原发性干燥综合征,1例确诊原发性干燥综合征3年后出现下肢血栓。3例均为女性,临床资料及实验室检查见表1、2、3。3例患者经华法令、肝素钙等处理在1～2周后患肢肿痛等临床症状缓解,之后长期服用阿司匹林（100mg／d）或华法令（1mg-1．5mg／d）治疗,例1患者因出凝血时间明显延长（国际标准化比值INR6．0）而停用华法令（1mg／d）后2周对侧患肢再发血栓。     

原发性干燥综合征59例皮肤黏膜表现分析

目的:了解原发性干燥综合征(pSS)患者皮肤黏膜表现,提高诊疗水平.方法:对59例pSS患者的临床资料进行回顾性分析.结果:59例pSS患者中,21例有皮肤黏膜表现,发生率为35.6％,其中表现为紫癜、瘀斑8例(38.1％),口腔溃疡4例(19.0％),皮肤干燥3例(14.3％),下肢红斑结节4例(19.0％),雷诺现象2例(9.5％).结论:pSS的皮肤黏膜表现并不少见,可能使患者直接到皮肤科就诊,所以皮肤科医生应了解pSS的相关知识并予以重视,这有助于pSS早期诊断,避免误诊和漏诊.     

氨苯砜超敏反应综合征5例临床分析

目的探讨氨苯砜超敏反应综合征的临床特征和治疗方法。方法回顾分析5例氨苯砜超敏反应综合征临床资料。结果5例氨苯砜超敏综合征用药潜伏期较长,为20～42天,均以发热或皮疹为首发症状,皮疹呈多形性,为麻疹样型、多形红斑型和红皮病型,所有患者均有浅表淋巴结肿大,肝肿大,1例脾肿大,并伴有血液学异常和肝功能受损,治疗时间平均58天,4例痊愈出院,1例死于肺部感染。结论氨苯砜超敏综合征表现为发热、皮疹、黄疸、淋巴结肿大、肝损害和溶血性贫血。根据DDS用药史,排除微生物感染和其他发疹性疾病可诊断本病,治疗应遵循重症药疹治疗原则。     

原发性和继发性干燥综合征67例临床分析

目的:探讨原发性和继发性干燥综合征(Ss)的不同临床特点,指导临床的诊断和治疗.方法:回顾性分析67例干燥综合征患者的临床症状、实验室及辅助检查结果和治疗的异同.结果:67例患者中41例为原发性Ss,26例为继发性Ss.原发性Ss常以单一症状发病,猖獗龋发生率、角膜荧光染色阳性率、γ-球蛋白升高发生率均较高,肾脏损害以肾小管间质损害为主;继发性Ss大部分以复合症状起病.肾脏损害以肾小球损害为主,肺损害较原发性Ss更严重.结论:原发性与继发性Ss有不同的临床特点和实验室检查结果,临床上要注意两者的鉴别和治疗的选择.     

植物日光性皮炎2例误诊分析

目的探讨植物日光性皮炎(phytophotodermatitis)误诊的原因与诊断应注意的问题。方法收集2例植物日光性皮炎误诊患者的临床资料,回顾分析其临床症状、诊断和误诊过程。结果 2例首诊均误诊。1例误诊为血管性水肿,1例误诊为卟啉病。结论对于春、夏季发病,有光敏性植物食用史及光曝露史,在曝露部位出现水肿及紫癜样皮疹者要首先考虑植物日光性皮炎。糖皮质激素能迅速控制症状、减轻痛苦,局部对症处理也是患者早日恢复的关键。     

高IgE综合征临床表现及与特应性皮炎的鉴别诊断

高IgE综合征(hyper-immunoglobulin E syndrome,HIES)是临床少见的以免疫和结缔组织紊乱为特点的原发性遗传免疫缺陷病,临床表现主要为慢性湿疹样皮炎,伴血清IgE水平及嗜酸粒细胞计数的持续升高、严重反复的细菌或病毒感染、骨骼发育异常等表现,部分患者早期有并发恶性肿瘤的可能。HIES属于少见病,临床医师对本病的认识不足,而特应性皮炎(atopic dermatitis,AD)和HIES的主要临床表现、实验室检查结果等有很多相似,HIES容易被漏诊或误诊为AD。该文通过归纳分析高IgE综合征的临床表现和实验室检查指标特征,比较与AD的异同,可提高临床医生对HIES的认识以及对这两种疾病的鉴别诊断能力。     

葡萄球菌性烫伤样皮肤综合征60例临床分析

对60例葡萄球菌性烫伤样皮肤综合征(SSSS)患儿的临床症状、皮疹特点及实验室检查进行回顾性分析.58例SSSS患儿红斑均首发于眼周、口周,并在眼周或口周红斑基础上出现糜烂、结痂,口周出现放射状皲裂;另有2例表现为躯干部泛发性大疱性脓疱,所有患儿尼氏征均阳性.眼周及口周红斑伴放射状裂纹对本病有特殊的诊断意义,治疗应早期足量应用抗生素.     

儿童过敏性紫癜诊治进展

过敏性紫癜是儿章时期常见的系统性血管炎,新的诊断标准将皮肤紫癜作为必要条件.因此,以腹痛为首发症状者早期较难诊断,而胃镜检查和凝血因子(ⅩⅢ)活性测定可提高本病早期诊断率,胰岛素样生长因子、胰岛素样生长因子结合蛋白3及肾尿表皮生长因子可以预测过敏性紫癜肾损害,为临床干预提供依据.过敏性紫癜治疗以药物为主,糖皮质激素和免疫抑制剂是治疗本病的有效药物并改善预后,麦考酚酯治疗本病前景广阔.     

Oropharyngeal Pemphigus Vulgaris and Clinical Remission

Background: The pattern of clinical remission in pemphigus vulgaris patients still remains a controversial issue because of the limited data reported in the literature. Objective: To evaluate the time to clinical remission in patients with exclusive oropharyngeal pemphigus vulgaris. Methods: We conducted a long-term, longitudinal study in a university hospital. We treated 37 patients with oropharyngeal pemphigus vulgaris, who underwent a periodic follow-up for an average of 5.3 years, and evaluated their outcome in terms of clinical remission. The main outcome measure was the clinical outcome (assessed by objective measures of severity, extent of disease, intensity of therapy, and remission) before and after conventional immunosuppressive therapy. Results: Complete and long-lasting clinical remission was achieved in 35 patients (94.6%) with oropharyngeal lesions, of whom 13 (35.1%) were off therapy and 21 (56.8%) were on therapy at the last evaluation. One patient (2.7%) died following a stroke 3 years after complete remission on therapy. Partial remission was achieved in two patients (5.4%). The mean time to achieve complete clinical remission was 4.7 ± 2.57 months after commencement of therapy. In all patients the mean disease severity score decreased from 7.81 ± 1.35 at time of diagnosis to 1.0 ± 0.9 at time of clinical remission (p < 0.0001 vs baseline), while the extent of the disease decreased from 2.9 ± 1.0 to 0.27 ± 0.45 (p < 0.0019 vs baseline) and the intensity of therapy from 4.91 ± 0.64 to 0.70 ± 0.57 (p < 0.0001 vs baseline). The mean duration of complete remission was 63.53 ± 44.9 months. Conclusions: In almost all patients with oropharyngeal pemphigus vulgaris it was possible to schedule a safe tapering of the conventional immunosuppressive therapy very shortly after the disease was controlled. Thus, we may conclude that: (i) the percentage of patients with oropharyngeal pemphigus vulgaris who achieved complete long-lasting clinical remission was very high; (ii) transient lesions that healed within a week were very frequent and had to be actively controlled; (iii) if treated early, most patients had a good clinical response and could achieve a disease- and drug-free clinical remission; (iv) early treatment may prevent extension or progression of disease; (v) there is a possible role for immunosuppressive agents; and (vi) a more favorable course of the disease, in terms of attainment and duration of clinical remission and a better prognosis, seemed to be related to a rapid response to therapy rather than to the initial severity and extent of the disease.     

Effects of isotretinoin therapy on lipoprotein (a) serum Levels

Background Increases in plasma concentrations of lipids, triglycerides, and liver enzymes have been reported in patients on isotretinoin therapy. Lipoprotein (a). (Lp (a)), a cholesterol-rich plasma lipoprotein, influences the clotting system and is related to premature coronary heart disease and stroke. Methods Blood (7 mL) was obtained from 30 patients with cystic acne before and 30 days after the initiation of oral isotretinoin (0.5 mg/kg/day). Results An increase in liver enzymes and lipids, except high density lipoprotein, was found in our patients at the end of the study. The mean Lp (a) levels (initial value, 25.91±3.17 mg/dL) were statistically reduced (p < 0.0001) at the end of treatment (14.80±2.35 mg/dL). Conclusions It is suggested that isotretinoin could be used as an Lp (a) lowering agent in the future.     

Henoch-Schönlein purpura associated with solid-organ malignancies: three case reports and a literature review

Adult Henoch-Sch?nlein purpura (HSP) is rarely associated with solid-organ malignancies. We describe here three adult patients with HSP diagnosed within 3 months of the diagnosis of associated solid-organ malignancies, including pulmonary, prostate, and renal carcinomas. Two patients had complete remission with a combination of immunosuppressive therapies and treatment of the associated malignancy. The third patient had partial remission with immunosuppressive therapies, but never received treatment for the associated malignancy and did not achieve complete remission before his death 10 months after diagnosis of HSP. These cases suggest that HSP associated with solid-organ malignancies may be resistant to immunosuppressive therapies without treatment of the associated malignancy. Therefore, evaluation for solid-organ malignancies should be considered in adult patients without an identifiable cause of HSP, especially if the disease is not self-limited or does not respond appropriately to treatment.     

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。     

Mycophenolate sodium for subacute cutaneous lupus erythematosus resistant to standard therapy

Background Approximately 75–95% of patients with cutaneous lupus erythematosus respond to antimalarial therapy and/or topical glucocorticosteroids. Immunosuppressive agents are usually considered a second‐line approach in patients with resistant disease. Objectives This was a prospective, nonrandomized, open pilot study to evaluate the efficacy of mycophenolate sodium monotherapy in patients with recalcitrant subacute cutaneous lupus erythematosus (SCLE). Methods Monotherapy with oral enteric‐coated mycophenolate sodium 1440 mg daily was given for a total of 3 months. Treatment outcome was evaluated by means of a validated clinical score for cutaneous lupus erythematosus, the Cutaneous Lupus Erythematosus Disease Area and Severity Index (CLASI), as well as 20‐MHz ultrasound measurements and colorimetry. Safety assessment included the monitoring of adverse effects and clinical laboratory parameters. Results Ten patients with active SCLE resistant to at least one standard therapy were included in the trial. Mycophenolate sodium led to a remarkable improvement of skin lesions, resulting in a significant decrease of the mean ± SD CLASI from 10·8 ± 6·0 at the beginning to 2·9 ± 2·6 at the end of therapy. Clinical improvement was confirmed by ultrasonographic assessments and colorimetry. No serious side‐effects were noted. Conclusions Mycophenolate sodium is beneficial and safe in the treatment of patients with SCLE that failed standard therapy. However, these preliminary data must be confirmed by randomized controlled trials including a larger sample size.     

老年HIV/AIDS住院患者48例流行病学及临床特征分析

目的探讨分析老年艾滋病(AIDS)病人的流行病学、临床特点以及治疗经验。方法回顾性研究2003-2009年重庆市公共卫生医疗救治中心收治的年龄在50岁及以上的老年艾滋病病毒(HIV)感染者/AIDS病人的临床数据资料,包括人口统计学、免疫学指标、病毒学指标、机会性疾病及HIV/AIDS诊断情况,以及老年抗病毒治疗情况等。结果 48例病人中,男42例,女6例;年龄平均(61.92±9.16)岁(50～92岁);38例经过性途径感染,2例经过同性性行为传播,另有2例经吸毒感染,6例不详。CD4+T淋巴细胞1～415/mm3,平均(83.33±105.00)/mm3;CD8+T淋巴细胞67～1741/mm3,平均(612.02±389.78)/mm3;病毒载量检测结果 :4例<50拷贝/mL,23例平均(5.87±0.86)Log10拷贝/mL(4.36～7.73Log10拷贝/mL)。机会性感染中,细菌性肺炎34例(70.83%),口腔念珠菌感染26例(54.17%),结核病15例(31.25%),耶氏肺孢子菌肺炎(PCP)感染14例(29.17%)。48例中有18例进行了抗病毒治疗。结论近年来,老年人群中HIV/AIDS问题不断凸现;其不易及时检出,机会性感染严重、合并症复杂、治疗效果延迟等现象是AIDS防控的棘手问题,应引起高度重视。     

Quality of life in patients with facial steroid dermatitis before and after treatment

Background Improper long‐term, even low‐dose, topical corticosteroids, especially application to the face, could induce steroid dermatitis, which was refractory and detrimental to the quality of life. Objective To evaluate the quality of life in patients with facial steroid dermatitis before and after the treatment of doxycycline and indomethacin plus support therapy. Study design A prospective study. Setting Outpatients of the Department of dermatology, the Third Hospital of Hangzhou, from August 2, 2004, to April 20, 2005. Subjects Fifty consecutive outpatients completed the treatment. Intervention The intervention is doxycycline 10 mg twice a day and indomethacin 25 mg twice a day for 4 weeks, cetirizine or loratadine 10 mg daily if pruritic, topical white petroleum if feeling dry and wet dressing if burning and oedema, plus psychological support and health education. Main outcome measure The efficacy of the treatment was quantified using a 24‐point steroid clinical score. The detriment of the quality of life was quantified using a 30‐point Dermatology Life Quality Index. Results The steroid dermatitis clinical score decreased significantly from 15.06 ± 4.61 at baseline to 4.52 ± 3.39 at 2 weeks after the end of treatment (week 6; P < 0.001). Twenty‐one patients underwent a rebound phenomenon and the steroid dermatitis clinical score increased significantly from 13.71 ± 4.33 at baseline (week 0) to 19.24 ± 3.40 at 1 week after treatment (week 1; P < 0.001). Quality of life score decreased significantly from 13.76 ± 7.68 at baseline to 3.44 ± 2.57 at 2 weeks after the end of treatment (week 6; P < 0.001). Conclusions The quality of life was profoundly affected by facial steroid dermatitis. Doxycycline and indomethacin plus support therapy might be effective in patients with facial steroid dermatitis.     

Comparison of immunological parameters in patients with pemphigus vulgaris following rituximab and IVIG therapy

Background Information on certain immunological parameters in patients with pemphigus vulgaris (PV) treated with rituximab (RTX) and intravenous immunoglobulin (IVIG) therapy is limited. Objective Comparing immunological parameters in patients who achieved long‐term clinical remission (LTR) with those who relapsed. Methods Retrospective analysis of 19 patients treated at a single centre using the same protocol. Comparisons were made between patients who went into LTR and those who relapsed following completion of the protocol. Treatments prior to IVIG and RTX included prednisone with or without an immunosuppressive agent. The immunological parameters measured included peripheral blood B cells (CD19+), serum quantitative immunoglobulin levels, and levels of antibodies to desmogleins (Dsg) 1 and 3. Results Eleven patients achieved LTR. Eight patients developed 15 relapses. The mean follow‐up time for the LTR group was 29·6 ± 11·2 months, and for the relapse group, 40·0 ± 7·0 months. There were no significant differences in times to B‐cell depletion, repopulation, or recovery to pretreatment levels between the patients who achieved LTR and those who relapsed. Recurrences usually occurred after B‐cell repopulation. Repeated treatments did not influence the time to B‐cell repopulation. IgM levels were decreased after therapy and remained decreased. A consistent increase in anti‐Dsg1 antibody levels occurred at the time of relapse in patients with mucocutaneous disease. Conclusions The majority of patients treated with rituximab and IVIG therapy achieved LTR. Retreatment of relapses can induce LTR. Decreased serum IgM levels persisted following treatment. Increases in anti‐Dsg1 antibodies during therapy in patients with mucocutaneous disease suggests a close follow‐up for a potential relapse is required.     

Various approaches to the length of time of outpatient observation of male patients with gonorrhea

The authors analyze the findings of clinical and laboratory examinations of 400 male patients with gonorrhea, carried out after the treatment, and correlate these findings with similar data obtained during the clinical diagnosis and therapy. In 288 subjects (62%) three-stage control examinations have yielded favourable results. This fact has called for the development of specific criteria for this patient population; it is suggested that a universal clinical and laboratory examination be carried out in 10-14 days after the treatment. Introduction of a universal reliable criterion of cure for gonorrhea patients will help cut down the periods of post-treatment follow-up.