Cicatricial Pemphigoid and Dry Eye

Cicatricial pemphigoid is the most common of the immunobullous disorders causing conjunctival cicatrization and is an autoimmune disease in which the ocular component of the immunopathology is directed at the conjunctival basement membrane. The disease is usually bilateral and more common in females, with most cases occurring between 30–90 years, and most often in the seventh decade. The disease occasionally occurs in children. Tear deficiency is a major cause of symptoms, although loss of vision is usually due to surface failure before the onset of aqueous tear deficiency, which occurs late in the progression of the disease. Management of the dry eye must be integrated with the management of the other components of both the ocular surface disease and inflammation. Management requires plastic surgery for the lid and lash malposition, tetracyclines and lid hygiene for the accompanying blepharitis. For the dry eye, the use of lubricants without preservatives is important, to avoid toxicity, and lubricant ointment is helpful for the relief of symptoms in terminally dry eyes without the capacity for surface wetting. Contact lenses, either large limbal diameter rigid gas permeable or gas permeable scleral lenses, are useful for treating dry eye and improving vision in some patients. Control of the conjunctival inflammation is mandatory to prevent disease progression and usually requires systemic immunosuppressive therapy.     

Incidence and Clinical Characteristics of Ocular Involvement in Mucous Membrane Pemphigoid

Purpose: To describe the risk of developing ocular mucous membrane pemphigoid (MMP) or a new extraocular site of MMP, and to identify risk factors for new involvement.

Methods: Retrospective chart review of 162 biopsy-proven MMP patients.

Results: At presentation, 109 of 162 MMP patients (67.3%) had ocular involvement and 53 patients did not. Of the 53 patients without ocular involvement at presentation followed up to 22 years, the risk of developing ocular MMP was 0.014 per person-year (PY, 95% confidence interval [CI]: 0.005/PY, 0.034/PY). The risk of developing any new location of extraocular MMP was 0.020/PY (95% CI: 0.007/PY, 0.043/PY). Smoking was a risk factor for developing an additional extraocular MMP location (hazard ratio [HR] = 4.09, p = 0.04).

Conclusions: Patients presenting with extraocular MMP are at risk for developing ocular MMP, and all MMP patients are at risk for developing secondary extraocular MMP locations, although the rates were low.     

兔大泡性角膜病变疼痛状态客观评价的方法学研究

背景 建立一种眼科疾病模型动物疼痛和痛苦的客观评价标准对于眼科基础和临床研究以及对实验动物的人文关怀都是非常必要的,目前国内尚缺乏这方面的研究. 目的 探讨定量评分法结合模型动物行为判断法评价大泡性角膜病变兔疼痛状态的有效方法. 方法 选取健康成年新西兰白兔12只,其中9只兔为实验组,均取左眼为实验眼,应用角膜内皮刮除术制作大泡性角膜病变模型,3只作为正常对照.于术前,术后1、3、7、14 d应用手持裂隙灯显微镜观察实验组模型兔角膜病变区的变化,应用超声生物显微镜(UBM)测量其中央角膜厚度(CCT),同时参考国际动物关怀与使用委员会(IACUC)起草的美国实验动物疼痛指南,通过建立“体质量+疼痛状态20项评分”法对疼痛指标进行量化评分,以评估实验动物的疼痛状态.结果 术后1d裂隙灯显微镜下观察可见9只大泡性角膜病变模型眼角膜明显水肿,呈灰白色混浊;术后3d角膜表面出现明显大泡,大泡破裂后角膜上皮糜烂,并持续到术后14d.术后1、3、7、14d模型眼CCT分别为(1468±100)、(2313±588)、(2391±271)、(2362±151) μm,与术前的(390±6) μm相比,差异均有统计学意义(均P=0.000),而术后3、7、14d间CCT比较差异无统计学意义(P＞0.05).术后1、3、7、14d实验动物体质量分别为(3.29±0.20)、(3.20±0.17)、(2.77±0.25)、(3.10±0.30)kg,与术前的(3.52±0.18)kg相比差异均有统计学意义(P=0.008、0.007、0.003、0.004).实验组术前和对照组所有实验兔在观察期内疼痛评分均为0分,实验组术后1、3、7、14d疼痛状态评分分别为7(7,7)、11(10,12)、9(8,10)、9(9,9)分,其中术后3d评分最高,与术后1、7、14 d相比差异均有统计学意义(P=O.007、0.005、0.007),与角膜出现大泡及角膜上皮糜烂时间基本吻合.20项指标中以进食减少、自我隔离/躲避、磨牙、进行抓持/点眼等操作时可观察到攻击性增加、活动性减少、姿势异常(头低位、弓背等)、发出低沉的叫声7项在所有9只实验动物术后均为阳性. 结论 应用“体质量+疼痛状态20项评分”法可以对大泡性角膜病变兔模型的疼痛状态进行有效、客观、量化的评价.     

大疱性表皮坏死松解型药疹致疹药物文献分析

目的 了解引发大疱性表皮坏死松解型药疹的药物致疹发生频率.方法 收集1989~2011年文献中有关大疱性表皮坏死松解型药疹的报道病例,对致疹药物进行统计,并分析致疹药物的不良反应发生率,推算大疱性表皮坏死松解型药疹的发生率.结果 统计表明,引发大疱性表皮坏死松解型药疹的药物品种有51种,阳性病例176例,抗感染药物涉及17种69例,神经精神类药物6种46例,解热镇痛药物4种28例.单一品种中卡马西平引发个案26例（发生率为5.04%）,氨基比林引发个案18例（发生率为0.68%）,苯巴比妥和阿莫西林各9例（发生率分别为1.24%和0.37%）.结论 引发大疱性表皮坏死松解型药疹的药物以抗生素类药物、神经精神类药物和解热镇痛类药物常见,致疹发生率较高的药物有卡马西平、苯妥英钠、别嘌醇和扑热息痛等.     

老年人大疱性类天疱疮与神经系统疾病关系的研究

目的探讨老年大疱性类天疱疮（BP）与神经系统疾病的关系。方法收集2008年1月至2010年10月在我院住院的23例老年BP（〉65岁）、11例成年BP（22～65岁）及23例老年其他皮肤病患者的临床数据进行回顾性病例对照分析。结果神经系统疾病在老年BP组出现的频率（43.5%）高于成年BP组（9.1%,P=0.06）和老年非BP组（8.7%,P〈0.01）,logistic回归显示具有神经系统疾病的老年人患BP的风险高于无神经系统疾病的老年人（OR=6.41,P〈0.05）。卧床仅发生于合并神经系统疾病的BP患者。BP患者所合并的神经系统疾病主要为痴呆、脑血管病和帕金森病。结论神经系统变性病和脑血管病是老年BP的独立危险因素,且BP与神经系统疾病的严重程度有关。神经系统损伤及交叉免疫反应可能在老年BP的发病过程中发挥重要作用。     

Detection of pemphigus desmoglein 1 and desmoglein 3 autoantibodies and pemphigoid BP180 autoantibodies in saliva and comparison with serum values

Although there is much literature on the detection of pemphigus and pemphigoid autoantibodies by enzyme-linked immunosorbent assay (ELISA) in serum, nothing is known about their presence in saliva. The aim of this study was to evaluate the salivary levels of these autoantibodies in pemphigus and pemphigoid patients. Autoantibodies against desmoglein3, desmoglein1, and BP180 were assayed, by ELISA, in serum and saliva samples of patients and healthy controls. The titres of autoantibodies against Dsg1/3 found in both serum and saliva of pemphigus patients showed a statistically significant correlation, suggesting that saliva may be a useful biological material for diagnostic purposes, in monitoring disease activity, as well as for the early detection of relapses. By contrast, the titres of autoantibodies against BP180 in the serum and saliva of bullous pemphigoid patients were not statistically related, and further study of the usefulness of the BP180 ELISA for saliva in this disease is needed. In addition, based on our results, the BP180 ELISA with a recombinant NC16a epitope failed to detect the autoantibodies against BP180 in the serum and saliva of mucous membrane pemphigoid patients.     

胸腔镜辅助小切口行肺大疱切除围手术期的护理

目的 分析总结对胸腔镜辅助小切口手术(vAMT)行肺大疱切除治疗自发性气胸的护理经验.方法 采用VAMT行肺大疱切除治疗自发性气胸37例患者.结果 全部手术获得成功,均治愈出院,无死亡病例.术后肺膨胀不全3例,肺部轻度感染2例,随访35例,占94.6%,无1例复发.结论 严密观察、精心护理,可提高VAMT行肺大疱切除治疗自发性气胸治愈率,减少并发症.     

Successful treatment of a severe persistent case of pemphigoid gestationis with antepartum and postpartum intravenous immunoglobulin followed by azathioprine

We report a case of pemphigoid gestationis affecting a 37-year-old woman in both her pregnancies. In her second pregnancy she failed to respond to high-dose oral prednisolone. She was treated successfully with intravenous immunoglobulin (2 g/kg each infusion cycle) as a steroid-sparing agent during both the antepartum and postpartum period. The baby was born at the 36th week of gestation. No complications were noted for either mother or baby. After ceasing intravenous immunoglobulin, azathioprine (up to a dose of 1 mg/kg/day) was used to adequately control her pemphigoid gestationis.     

Bullous Variant of Pyoderma Gangrenosum in a Patient with Acute Myeloid Leukemia

Unlike classic pyoderma gangrenosum (PG), the bullous variant of PG is typically represented by a painful erythematous papule, plaque, and superficial bulla that progress into the ulceration with bullous margin. Generally, bullous PG is most commonly associated with myeloproliferative disorders, such as acute myeloid leukemia (AML). Bullous PG in AML patients rarely occurs, but once it does, it suggests a poor clinical prognosis. Although many cases of classic PG in AML patients have been reported, bullous PG is relatively rare. Therefore, we present a case of bullous PG that developed in a patient with AML and was successfully treated with high-dose systemic steroids.     

Indications for penetrating keratoplasty in East China, 1994–2003

Purpose To evaluate the indications for penetrating keratoplasty (PKP) and associated procedures in the Eye and ENT Hospital, Fudan University, Shanghai, People’s Republic of China, an eye center in East China and to compare our results with those from previous studies. Methods We retrospectively studied the charts of cases who had received PKP in the hospital from 1994 to 2003. The recorded data covered age, sex, and preoperative clinical diagnoses of PKP, and surgical procedures in some cases. Results A total of 229 cases received PKP during that period. The number of surgical cases increased dramatically in the last 3 years (2001–2003). The leading indication for PKP was corneal scar (55.9%), which included 44 (19.2%) cases of corneal scar after viral keratitis and 30 (13.1%) cases of corneal scar after chemical/thermal burn. Other indications, in order of decreasing frequency, were bullous keratopathy (12.2%), acute necrotizing/ulcerative keratitis (12.2%), regraft (10.5%), keratoconus (5.7%), and non–Fuchs’ corneal dystrophies (3.8%). Combined procedures were performed on 22 cases. The most three common indications for PKP, corneal scar, bullous keratopathy, acute necrotizing/ulcerative keratitis, showed an increasing frequency in the period of the last 3 years. Conclusion The leading indication for PKP was corneal scar, which differed from the conclusion offered by the developed areas in the world, where bullous keratopathy or keratoconus was the case. A similar result was also found in reports from India and Taiwan.