大疱性类天疱疮患者血清鳞状细胞癌抗原的检测

目的探讨血清鳞状细胞癌抗原(squamous cell carcinoma antigen,SCCA)水平与大疱性类天疱疮(bollous pemphigoid,BP)的关系。方法采用回顾性病例对照研究,根据设定标准选择BP患者,收集临床资料。采用微粒酶免疫分析法测定血清SCCA值,大于1.50ng/mL为阳性。根据血清SCCA水平分为阴性组和阳性组。对两组间患者皮损面积(其中面积评分0～6分)、严重程度(0～3分)、总体评分(面积评分和严重程度评分的乘积)等指标差异进行比较及统计学分析。结果共入选23例BP患者,SCCA阳性率52.20%,升高范围1.80～70.00ng/mL。阴性、阳性两组间面积评分、总体评分差异有显著性。面积评分及总体评分各自分为两组分别计算比值比均显著大于1。等级相关分析结果显示总体评分和SCCA水平相关系数为0.668,P0.001。结论 SCCA在BP患者中可以升高,且SCCA水平和BP疾病严重程度呈正相关,它对制定治疗方案、判断疗效有一定参考意义。     

天疱疮和类天疱疮患者生活质量评定及影响因素分析

目的评价天疱疮和类天疱患者的生活质量,探讨影响其生活质量的相关因素。方法选择本科门诊84例大疱病患者(天疱疮44例,类天疱疮40例),并以银屑病患者作为对照组,采用世界卫生组织生活质量测定量表简表(WHOQOL-BREF)、皮肤病生活质量指数(DLQI)及简明健康调查问卷(SF-36)对寻常性银屑病、天疱疮和类天疱疮三组患者的生活质量进行评定,并对影响其生活质量的相关因素进行分析。结果天疱疮组和类天疱疮组WHOQOL-BREF总体感受得分低于银屑病组,差异有统计学意义(P0.05);天疱疮组和类天疱疮组与银屑病组DLQI总分比较,差异无统计学意义(P0.05);类天疱疮组在生理机能领域的得分低于天疱疮组,天疱疮和类天疱疮患者中皮损累及皮肤黏膜者在社会功能领域的得分低于累及皮肤者,疾病重度者在生理职能和情感职能领域的得分低于轻、中度者,有合并症者在生理机能和情感职能领域的得分低于无合并症者,差异均有统计学意义(P均0.05)。结论天疱疮和类天疱疮患者较银屑病患者生活质量低,疾病类型、皮损分布、疾病严重度和有无合并(并发)症对两种大疱病患者的生活质量有明显的影响。     

大疱性皮肤病48例临床分析

目的总结大疱性皮肤病的临床特点和治疗经验。方法对48例大疱性皮肤病住院患者的临床表现、实验室检查结果和临床疗效进行回顾性分析。结果本研究中48例大疱性皮肤病患者中有28例患者为大疱性类天疱疮,占总数的58.3%。大疱性类天疱疮患者中20例单独使用糖皮质激素治疗的患者皮损痊愈8例,好转11例,治愈率40%;糖皮质激素联合丙种球蛋白（IVIG）治疗的4例患者中,3例达到皮损痊愈,治愈率75%。大疱表皮松解型药疹患者中4例单独使用糖皮质激素治疗的患者皮损痊愈2例,好转2例,治愈率50%;糖皮质激素联合丙种球蛋白（IVIG）治疗的6例患者中,4例达到皮损痊愈,1例好转,治愈率66.7%。结论本研究提示大疱性类天疱疮是大疱性皮肤病中最常见的一类疾病;糖皮质激素在大疱性皮肤病治疗中的疗效肯定,且注射丙种球蛋白是一种重要的支持治疗方法。     

Bullous pemphigoid induced by vildagliptin: a report of three cases

To report three cases of bullous pemphigoid in patients treated with vildagliptin. Case 1: An 86‐year‐old woman presented with bullous pemphigoid after 1 month of treatment with vildagliptin and metformin. After introduction of clobetasol, the symptoms resolved although vildagliptin was continued. However, the skin lesions reappeared 3 months later. Sustained remission was achieved only after definitive withdrawal of vildagliptin. Case 2: A 79‐year‐old man presented with bullous pemphigoid after 37‐month treatment with gliclazide, vildagliptin and metformin. The disease at first responded to clobetasol but 3 months later the lesions reappeared. They finally regressed when the gliptin was discontinued. Case 3: A 77‐year‐old woman, treated with gliclazide and vildagliptin for 26 months, presented with bullous pemphigoid, which responded well to discontinuation of the gliptin and topical clobetasol. Gliptins are new molecules for treatment of type 2 diabetes mellitus, which have been suspected of implication in bullous pemphigoid. Such cases have been described in the literature (seven with vildagliptin and three with sitagliptin). In nine of these cases, the gliptin was associated with metformin, but the latter had never been considered responsible. The mechanism implicated in the development of bullous pemphigoid has not yet been clearly identified, but may involve a modified immune response or alteration of the antigenic properties of the epidermal basement membrane. These reports support the risk of bullous pemphigoid in patients exposed to gliptins.     

The Effect of Donor- and Recipient-Related Factors on Corneal Graft Survival in Penetrating Keratoplasty

Purpose: To investigate the impact of donor and recipient factors on graft survival in penetrating keratoplasty (PK).

Material and Methods: This retrospective study included 365 eyes that underwent PK using corneas from 231 donors between June 2010 and June 2015. Patients were divided into three groups (group 1: primary endothelial diseases; group 2: iatrogenic endothelial disorders; and group 3: other pathologies with a healthy endothelium) according to PK indications. The primary outcome measure was corneal graft survival at the last visit (clear or opaque). Graft clarity was assessed using Kaplan–Meier survival analysis.

Results: The most frequent PK indication was keratoconus (KC) (20.5%) followed by pseudophakic bullous keratopathy (PBK) (18.9%). Donor age had a negative impact on endothelial cell density (ECD) measured by an eye bank specular microscope (p < 0.001). Median best-corrected visual acuity in logarithm of the minimum angle of resolution units increased from 2.1 to 0.8 at 1 year after PK (p < 0.001). The clear graft rate was 96.7% at year 1, 88.8% at year 2, and 85.5% at year 3. Overall graft survival was 84.9% during a median of 39 months (range: 24–79 months) of follow-up. A higher graft survival rate (67.2%) was observed in KC compared to PBK during 6 years (p < 0.001). Recipients younger than 50 years of age showed a better graft survival rate than those older than 70 years of age (p = 0.037). Donor ECD, time between excision and death, and preservation time had no significant effect on graft survival. Frequent graft rejection episodes (GREs) and additional procedures during surgery had a negative impact on graft survival (p < 0.001 and p = 0.014, respectively). A worse graft survival was observed in group 2 compared to groups 1 and 3 (p = 0.042).

Conclusions: Young recipient age and KC were associated with a better graft survival. Graft endothelial density and preservation time had no impact on graft survival. PBK, low vision at baseline and year 1, frequent GREs, and additional interventions during surgery had a negative impact on graft survivals.     

Role of physical factors in the pathogenesis of bullous pemphigoid: Case report series and a comprehensive review of the published work

Bullous pemphigoid (BP) is an autoimmune subepidermal blistering disease affecting mainly the elderly. The subtype of the disease induced by physical agents represents a rare and, therefore, insufficiently characterized form. In the present study, we aimed to contribute to a better understanding of the pathogenetic mechanisms involved in the onset of BP induced by different trigger factors. We have retrospectively analyzed nine cases of BP. All patients were characterized based on clinical, epidemiological and immunological parameters. For each case, the trigger factor involved was specified. In addition to our retrospective analysis, a comprehensive review of the 59 published cases was conducted, regarding the involvement of trigger factor in BP, and clinical, epidemiological and immunological data were collected. In the local study, conducted on nine patients diagnosed with BP, various trigger factors were identified: contrast substance injection, surgical procedure, mechanical trauma, insect bite, thermal burn, radiotherapy and ultraviolet exposure associated with pre‐existing psoriasis. The autoantibodies from all patients were shown to activate granulocytes and induce dermal–epidermal split. Different hypotheses regarding the pathogenetic mechanism involving the trigger factors have been discussed. In regard of the pathogenetic mechanism, we believe that the most reliable hypothesis is that BP patients already have low titers of anti‐basement membrane autoantibodies which activate the granulocytes. However, more studies are needed for a better understanding of the pathogenetic mechanism of the intervention of trigger factors.     

Case of pemphigus with immunoglobulin G and A antibodies,binding to both the intercellular spaces and basement membrane zone

We report a case involving a 62‐year‐old woman with in vivo‐bound immunoglobulin (Ig)G and IgA antibodies in both the intercellular space (ICS) and basement membrane zone (BMZ). Her clinical and histopathological features were identical with those of pemphigus vulgaris, while the immunopathological findings suggested IgG/IgA pemphigus. Direct immunofluorescence (IF) showed in vivo‐bound IgG and IgA antibodies in the ICS and BMZ, whereas indirect IF showed circulating IgG but not IgA antibodies in the ICS and BMZ. The anti‐ICS IgG bound to desmoglein‐3, while the anti‐BMZ antibodies bound to the epidermal side of 1 mol/L NaCl‐split skin. To the best of our knowledge, only two similar cases have been reported so far. Furthermore, we also examined IgG subclass distribution of the in vivo‐bound and circulating anti‐ICS and BMZ antibodies, and found that IgG1, IgG2 and IgG4 bound to ICS of the lesional skins, while IgG1 and IgG3 bound to the BMZ. The circulating anti‐ICS antibodies belonged to IgG1 and IgG4, while the circulating anti‐BMZ antibodies to IgG1, IgG2 and IgG4. We report a case involving a 62‐year‐old woman with in vivo‐bound immunoglobulin (Ig)G and IgA antibodies in both the intercellular space (ICS) and basement membrane zone (BMZ). Her clinical and histopathological features were identical with those of pemphigus vulgaris, while the immunopathological findings suggested IgG/IgA pemphigus. Direct immunofluorescence (IF) showed in vivo‐bound IgG and IgA antibodies in the ICS and BMZ, whereas indirect IF showed circulating IgG but not IgA antibodies in the ICS and BMZ. The anti‐ICS IgG bound to desmoglein‐3, while the anti‐BMZ antibodies bound to the epidermal side of 1 mol/L NaCl‐split skin. To the best of our knowledge, only two similar cases have been reported so far. Furthermore, we also examined IgG subclass distribution of the in vivo‐bound and circulating anti‐ICS and BMZ antibodies, and found that IgG1, IgG2 and IgG4 bound to ICS of the lesional skins, while IgG1 and IgG3 bound to the BMZ. The circulating anti‐ICS antibodies belonged to IgG1 and IgG4, while the circulating anti‐BMZ antibodies to IgG1, IgG2 and IgG4.