23例中重度大疱性类天疱疮皮质类固醇激素用量分析

回顾分析23例中重度大疱性类天疱疮患者的临床资料,探讨中重度大疱性类天疱疮合理的治疗方案。结果：中度大疱性类天疱疮患者皮质类固醇激素控制量（相当于泼尼松剂量）为（57．43±11．58）mg／d,重度患者控制量为（87．26±14．52）mg／d。联合免疫抑制剂治疗,可减少皮质类固醇激素的用量,利于其较快减量,减少激素使用副作用的发生。     

大剂量免疫球蛋白合并泼尼松治疗重症寻常型天疱疮1例

大剂量免疫球蛋白静脉滴注（ＩＶＩｇ）已被用于多种自身免疫性疾病的治疗［１］．最近我们对１例大剂量皮质类固醇激素与环磷酰胺并用不能控制病情且出现严重副作用的寻常型天疱疮患者加用ＩＶＩｇ治疗获得成功，报告如下。１临床资料患者女，５７岁。因口腔糜烂７月，四肢及躯干起水疱３月，加重１周入院。７月前患者无明显诱因口腔糜烂、疼痛，按口腔扁平苔藓治疗无效，病情渐加重；３月前胸、背、腋窝及腹股沟出现散在的水疱，易擦破，在当地医院诊断为天疱疮，予以泼尼松６０ｍｇ／ｄ，病情得到控制，泼尼松渐减量。入院前１周泼尼松减…     

狼疮性肾炎并发带状疱疹临床分析

１９９０年～１９９６年初在我院住院的狼疮性肾炎（ＬＮ）并发带状疱疹（ＨＺ）患者共１１例，现报告如下。一般情况５年间共收治ＬＮ３６例，发生ＨＺ１１例，占３０．６％，女性多见。多发生在使用大剂量强的松、甲基强的松龙或大剂量环磷酰胺（ＣＴＸ）冲击治疗过程中...     

黏膜性类天疱疮68例临床回顾分析北大核心CSCD

目的总结1987年8月至2012年10月我科诊治的68例黏膜性类天疱疮的临床特点和治疗经验。方法总结黏膜性类天疱疮患者黏膜皮肤损害、组织病理改变、免疫学等方面的特点,重点分析治疗方案。结果黏膜性类天疱疮68例,累及口腔黏膜67例占98．5％,眼部黏膜23例占33．8％。免疫学检查：16例行直接免疫荧光检查（DIF）,8例阳性;39例行间接免疫荧光检查（IIF）,8例阳性,阳性率20．5％;41例采用ELISA法检测血清中抗BP180抗体,22例阳性,阳性率53．7％。依据皮损分布确定病情严重程度后,予以局部治疗5例;中低剂量糖皮质激素系统治疗（0．4—0．5mg·kg^-1·d^-1,或30mg／d）55例,其中3例同时累及皮肤、口腔黏膜、眼结膜及其他黏膜的患者剂量增加至50mg／d左右才能控制病情。控制剂量起效时间（11．80±5．88）d,控制剂量服药时间0．23～12（3．06±2．84）个月。15例患者基本治愈,其中4例完全治愈。基本治愈患者糖皮质激素剂量减至原剂量50％所需时间为（13．29±5．76）个月,达到基本治愈所需时间为（17．33±7．71）个月。病情控制6个月后,剂量平均减至控制剂量的76．5％,12个月后剂量平均减至控制剂量的58．1％。3例患者在治疗过程中出现口腔念珠菌感染。结论黏膜性类天疱疮的诊断目前主要依据典型的临床和病理表现,尚缺乏灵敏度高的免疫学检查手段。中低剂量糖皮质激素系统治疗,配合局部治疗,常可达满意疗效。     

大剂量氨溴索联合气管镜治疗高位截瘫患者肺部感染临床研究

目的观察大剂量氨溴索联合纤支镜在治疗高位截瘫患者合并肺部感染的临床效果。方法合并严重肺部感染的高位截瘫患者30例,随机分成3组：小剂量氨溴索组（氨溴索30mg,壶入3次／d）;大剂量氨溴索组（氨溴索300mg,壶入3次／d）;联合治疗组（氨溴索300mg,壶入3次,d）,联合纤支镜下吸痰治疗。比较各组患者治疗前以及治疗第3d,第7d的临床效果、体征、X线表现、血气分析及呼吸机使用时间等。结果大剂量组患者在临床效果、体征及X线表现好转程度明显优与小剂量组（均P〈0．05）,且感染控制及脱机时间均显示缩短。联合治疗组较大剂量组治疗效果更加明显。结论对高位截瘫合并肺部感染患者,应用大剂量氨溴索可以有效化痰,控制感染,减少控制感染时间,减少呼吸机辅助呼吸时间,联合纤支镜治疗效果更好。     

大剂量盐酸氨溴索治疗急性百草枯中毒肺损伤的临床观察

目的观察大剂量盐酸氨溴索治疗急性百草枯中毒（PQ）肺损伤的疗效。方法将41例急性百草枯中毒肺损伤患者分为治疗组（21例）和对照组（20例），对照组给予大剂量甲泼尼龙、抗氧化以及血液净化等治疗，使用呼吸机通气；治疗组在对照组治疗基础上加用盐酸氨溴索注射液20mg·kg^-1·d^-1静脉滴注q12h，3-5d．观察两组动脉血气、呼吸参数，X线胸片变化（第1、3、5天），发生多脏器功能障碍综合征（MODS）（≥3个脏器）及多器官衰竭（MOF）病例，病死率与死亡病例存活时间。结果治疗后两组氧分压、氧合指数以及自主呼吸频率与潮气量、呼气末正压、X线胸片改变等均有改善（P〈0．01），治疗组的改善与对照组比较改善更为显著（P〈0．05）。两组间MODS（≥3）与MOF、病死率与死亡病例存活时间差异无统计学意义（均P〉0．05）。结论大剂量盐酸氨溴索注射液虽未能逆转患者的整个病程，但可以显著改善动脉血气、呼吸参数，对急性PO中毒肺损伤有积极的治疗作用。     

静脉注射丙种球蛋白治疗重型水痘的治疗体会

重型水痘常见于年长儿童和青年人，其临床症状重，病程较长，可合并肺炎、心肌炎等。试用静脉注射丙种球蛋白（ＩＶＩＧ）治疗８例重型水痘，表明单次剂量２．５ｇ安全有效，使症状迅速缓解，病程明显缩短。     

甲泼尼龙联合环孢素A冲击治疗对口服百草枯中毒致肺纤维化的早期干预研究

目的通过回顾性对照研究,观察采用大剂量甲泼尼龙联合环孢素A早期冲击治疗对百草枯中毒患者肺纤维化发生率及病死率的影响。方法将2004年1月～2007年11月收治的151例口服百草枯中毒患者分为对照组（50例）和试验组（101例）;所有患者均给予常规洗胃、血液灌流及补液等对症支持治疗;对照组中氧合下降、胸部CT提示肺纤维化者采用甲泼尼龙（15mg／kg）＋环磷酰胺（15mg／kg）冲击治疗;试验组患者来诊后立即使用甲泼尼龙（15mg／kg）＋环孢霉素A（5mg／kg）冲击治疗;比较2组患者的病死率、肺纤维化发生率及因肺纤维化而死亡者占各组患者总数的百分比。结果2组患者的年龄、服毒量、重度中毒患者占各组患者总数的百分比三项指标比较差异无统计学意义;对照组和试验组的病死率分别为52．00％（26／50）和43．56％（44／101）,差异无统计学意义（P=0．328）;肺纤维化发生率分别为20．0％（10／50）和6．93％（7／101）,差异显著（P=0．027）;因肺纤维化而死亡者占各组患者总数的百分比分别为18．00％（9／50）和4．95％（5／101）,差异显著（P=0．015）。结论采用大剂量甲泼尼龙联合环孢素A早期冲击治疗可显著降低百草枯中毒致肺纤维化的发生率及病死率,但其确切效果尚需通过大样本的多中心临床随机、对照研究进一步明确。     

适时机械通气策略与大剂量氯磷定在重度有机磷中毒所致急性呼吸衰竭中的临床应用评价

目的探讨适时机械通气策略在重度有机磷中毒（AOPP）所致急性呼吸衰竭（ARF）的临床应用价值。方法自2003年3月至2007年8月使用适时机械通气策略治疗重度AOPP所致ARF患者21例为治疗组，以适时机械通气为治疗重点，在维持阿托品化基础上，给予大剂量氯磷定。选择1998年5月至2002年10月采用常规方法治疗重度AOPP所致AHF患者19例作为对照组，观察两组机械通气时间、住ICU时间、氯磷定剂量、阿托品用量、中间综合征（IMS）发生率、救治成功率。结果治疗组机械通气时间为（115．3±86．2）h，显著短于对照组[（168．4±110．7）h，P〈0．051。治疗组住ICU时间为（12．1±4．5）d，显著短于对照组[（18．3±9．6）d，P〈0．05]。治疗组3d内氯磷定总量（20．2±2．0）g，显著大于对照组[（8．0±3．0）g，P〈0．01]。治疗组中间综合征发生率（23．8％，5／21），显著少于对照组[52．6％（10／19），P〈0．05]。治疗组阿托品用量为（292．5±115．7）mg，显著低于对照组[（570．3±150．2）mg，P〈0．011。治疗组抢救成功率为85．7％（18／21），显著高于对照组[47．4％（9／19），P〈0．01]。结论适时机械通气策略并大剂量氯磷定治疗AOPP所致ARF疗效显著。     

慢性荨麻疹患者血清IL-4、IFN-γ及IgE水平观察

测定３８例慢性荨麻疹（其中１２例为人工性荨麻疹）患者血清ＩＬ－４、ＩＦＮ－γ及ＩｇＥ水平，结果患者组ＩＬ－４高于对照组（正常人，Ｐ〈０．０１），患者组ＩＦＮ－γ低于对照组（Ｐ〈０．０５），患者组ＩｇＥ水平高于对照组（Ｐ〈０．０１）。     

糖皮质激素联合静脉用免疫球蛋白治疗老年皮肌炎2例

采用中等剂量糖皮质激素联合大剂量静脉注射用免疫球蛋白(IVIg)冲击治疗2例有糖皮质激素应用相对禁忌、且心肌酶谱非常高的老年皮肌炎患者。1例患者IVIg剂量为300mg/(kg·d),共5d,1例患者IVIg剂量为400mg/(kg·d),共4d。2例患者经治疗肌肉症状迅速缓解,心肌酶谱值在短期内降至正常,治疗中无明显不良反应。提示中等剂量糖皮质激素联合大剂量IVIg冲击治疗老年皮肌炎具有良好的疗效。     

Treatment of vitiligo with oral corticosteroids.

17 patients with generalized vitiligo and 5 patients with localized vitiligo were treated with oral corticosteroids. Six patients with generalized vitiligo showed over 75% repigmentation in at least one of the patches. Two patients with generalized vitiligo and 1 patient with localized vitiligo showed a partial repigmentation of 25-75%, while 5 patients with generalized vitiligo and 1 patient with localized vitiligo showed a minimal repigmentation of less than 25%. Four patients with generalized vitiligo and 3 patients with localized vitiligo failed to respond. Repigmentation became evident within 4 weeks in most cases, and in general was more markedly noted in exposed areas.     

Serum lysozyme in patients with localized and generalized granuloma annulare

Serum lysozyme (Muramidase) levels in patients with localized and generalized granuloma annulare were measured by a turbidometric method. More lysozyme is present in the serum samples of patients with generalized granuloma annulare than patients with the localized form or normal controls. The mean level of patients with generalized disease was 9.27 mg/L compared with 5.96 mg/L for patients with localized disease and 6.8 mg/L for controls.     

A study of HLA antigen association in localized and generalized granuloma annulare

HLA-A and -B antigen frequencies were studied in 78 patients, 35 with localized granuloma annulare and 43 with generalized granuloma annulare (GA). Twenty-eight patients in each group were also typed for HLA-DR antigens. A group of 200 healthy age-matched subjects served as controls. HLA-A31 and B35 were increased significantly in patients with generalized GA, but not in the localized form. HLA-DR antigen distribution showed no significant variation.     

Therapeutic success of epidermal grafting in generalized vitiligo is limited by the Koebner phenomenon

Eighteen patients with vitiligo (two with localized type, five with segmental type, and 11 with generalized type) were treated with an epidermal graft using the roof of a suction blister. In all patients with localized and segmental lesions and in seven with generalized lesions, successful repigmentation without scarring was observed at the grafted sites approximately 2 to 3 weeks after grafting, and a subsequent two- to threefold expansion of the pigmented area occurred within 3 to 4 months. On the other hand, no repigmentation could be achieved in four patients with generalized vitiligo in whom depigmentation (i.e., the Koebner phenomenon) occurred at the donor sites. Our results suggest that the Koebner phenomenon occurs in an all-or-none pattern only in patients with generalized vitiligo. Thus, although epidermal grafting is a simple and useful method, successful repigmentation at the recipient sites depends on the selection of patients with localized lesions in whom the Koebner phenomenon does not develop.     

Different phenotypes of segmental vitiligo based on a clinical observational study

Background Segmental vitiligo and generalized vitiligo are in general considered separate entities. However, clinico‐epidemiological data on segmental vitiligo are scarce compared with those of generalized vitiligo. Objective To analyse the clinical profile and distribution pattern of lesions in segmental vitiligo patients. Methods Segmental vitiligo patients were examined and questioned in a prospective and retrospective setting. The distribution and extent of the lesions were evaluated using clinical photographs. Results Different phenotypes of segmental vitiligo were found, including the unilateral segmental type (124 patients; group 1), the bilateral segmental type (three patients; group 2) and the mixed segmental and generalized type (14 patients; group 3). Furthermore, lesions were present with (10%) or without associated halo naevi. The age of onset of segmental vitiligo (median 14 years) was significantly different between the three subgroups (P = 0.028). Extensive involvement of segmental vitiligo lesions on trunk and extremities was significantly (P = 0.031) more observed in patients with a lower age of onset, while the generalized vitiligo lesions in the mixed vitiligo group were mostly very mild. Associated autoimmune diseases were reported in 11%, whereas a positive family history for vitiligo was present in 14.9% of patients. Lesions were not strictly dermatomal nor Blaschkolinear, although a typical recurring pattern could be observed. Conclusion Our data provide clinical evidence that segmental vitiligo and generalized vitiligo are parts of the same disease spectrum and that segmental vitiligo could have a polygenetic background as well. Whether different aetiopathological mechanisms underlie the different clinical phenotypes of segmental vitiligo remain to be elucidated.     

Acute generalized exanthematous pustulosis: analysis of cases managed in a tertiary hospital in Singapore

Acute generalized exanthematous pustulosis We reviewed eight cases of acute generalized exanthematous pustulosis managed in a tertiary hospital in Singapore. Background Acute generalized exanthematous pustulosis (AGEP) is a pustular reaction characterized by a sudden eruption of widespread nonfollicular sterile pustules associated with systemic symptoms. AGEP is primarily believed to be an adverse reaction to drugs. Outside of Europe, few reports exist on the clinical presentation of AGEP. Methods  A retrospective review of patients who presented with AGEP to a Singapore tertiary hospital was performed. Results Eight patients were diagnosed with AGEP in 2006–2007. All patients presented with a macular, maculopapular eruption prior to the onset of pustules. AGEP was characterized by nonfollicular, pinpoint pustules, which were generalized and accentuated in the flexural areas in seven of the patients. The remaining patient had erythematous patches, and scattered plaques studded with pustules. Other less common features included oral/genital mucositis, blisters and erosions. The mean duration from the cessation of medications to defervescence, resolution of pustules and normalization of leukocytosis was 5, 6, and 7 d, respectively. Despite the resolution of pustules, two patients had protracted clinical courses with evolution to generalized exfoliative dermatitis and Drug rash, eosinophilia, and systemic symptoms (DRESS), respectively. The implicated medications included antibiotics in six patients, morphine and phenytoin in one patient each. Conclusions Acute generalized exanthematous pustulosis is generally described as benign and self‐limiting. However, in certain patients, the clinical course is prolonged and may exhibit features that overlap with other forms of cutaneous adverse drug reactions such as toxic epidermal necrolysis and DRESS.     

环状肉芽肿43例分析

目的 探讨局限型、泛发型环状肉芽肿诱发因素、临床、组织学改变及治疗情况。方法 回顾性分析24例局限型及19例泛发型环状肉芽肿患者临床资料。结果 两型中尤其是泛发型中部分患者病情与日晒明显相关。局限型皮损常局限于手、颈后、足背，小丘疹排列成环状，直径1～2cm，最大7cm；泛发型皮损较小，0．5～1cm，常泛发。组织学改变以栅状肉芽肿最多见，占61．9％，散在性组织细胞浸润次之，占38．1％．两者均以局限型较泛发型为多。局限型以避光、局部外用糖皮质激素、冷冻、手术切除为主．必要时口服维生素E、烟酰胺、顽固者口服小剂量氯喹可控制病情。泛发型以系统用药为主，氯喹、氨苯砜、糖皮质激素及异维A酸可取得满意疗效，但部分停药后易复发。结论日光照射可能在泛发型环状肉芽肿发病中起着一定的作用；组织学改变以栅状肉芽肿最多见；局限型以局部治疗为主，泛发型以系统治疗为主。     

特殊类型银屑病伴发高尿酸血症相关因素分析

目的:探讨特殊型银屑病伴发高尿酸血症的相关因素。方法:对117例红皮病型、泛发性脓疱型或/及关节病型银屑病患者的临床资料进行回顾性分析;根据尿酸代谢过程、影响因素、银屑病的临床表现等确定分析指标15项,对高尿酸血症组、正常组间各指标先后进行单因素分析及多因素Logistic回归分析;同时分析特殊用药、病情演变与患者血尿酸水平变化间的关系。结果:特殊类型银屑病伴高尿酸血症发生率为31.62%;高尿酸血症组与正常组比较,性别、红皮病型损害、泛发性脓疱型损害及血甘油三酯增高间的差异均有统计学意义(P<0.05);红皮病型损害、男性、甘油三酯增高、泛发性脓疱型损害是影响高尿酸血症发生的重要因素,其相对危险度(RR)依次为8.93、6.83、2.86、0.31。结论:特殊型银屑病伴发高尿酸血症可能与性别、红皮病型损害、泛发性脓疱型损害、甘油三酯增高及特殊用药有关。     

Soluble CD4 and CD8 in serum from patients with localized scleroderma

Localized scleroderma has been shown to be accompanied by various immunologic abnormalities. To obtain functional information on activated CD4+ or CD8+ T cells, we studied the levels of soluble CD4 (sCD4) and soluble CD8 (sCD8) in serum from patients with localized scleroderma. Serum samples were examined by enzyme-linked immunosorbent assay. The samples were obtained from 49 patients in the following three subgroups: 15 patients with generalized morphea, 22 with linear scleroderma, and 12 with morphea. The levels of sCD4 and sCD8 were significantly elevated in patients with generalized morphea. Furthermore, these patients showed significantly higher levels of sCD4 than those with systemic sclerosis (SSc). The frequency of positivity for IgG anti-single-stranded DNA (ssDNA) antibody was significantly higher in localized scleroderma patients with elevated sCD4 levels than in patients with normal sCD4 levels. The frequency of positivity for antinuclear antibodies, IgM antihistone antibodies, IgG anti-ssDNA antibody and rheumatoid factor, and elevated sCD23 levels were significantly higher in localized scleroderma patients with elevated sCD8 levels than in patients with normal sCD8 levels. Our findings suggest that both CD4+ and CD8+ T cells are activated in vivo in generalized morphea and that the immunologic events in generalized morphea are different from those in SSc.