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Recalcitrant oral involvement in pemphigus vulgaris successfully treated with platelet-rich plasma. 用富血小板血浆成功治疗难治性寻常型天疱疮。
IF 2 Q3 Medicine Pub Date : 2020-10-16 DOI: 10.23736/S0392-0488.20.06664-X
F. Bardazzi, L. Sacchelli, R. Balestri, F. Filippi, A. Patrizi, Ivano Iozzo, C. Loi
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引用次数: 0
Paraneoplastic autoimmune multiorgan syndrome (PAMS). 副肿瘤自身免疫多器官综合征(PAMS)
IF 2 Q3 Medicine Pub Date : 2020-10-16 DOI: 10.23736/S0392-0488.20.06675-4
D. Didona, G. Di Zenzo, P. Joly
Originally described by Anhalt as paraneoplastic pemphigus in 1990, paraneoplastic autoimmune multiorgan syndrome (PAMS) is a potentially lethal blistering disease, characterized by polymorphous clinical features, including mucocutaneous erosions, blisters, lichenoid papules, and erythema. Several autoantibodies have been detected in serum of PAMS patients, including anti-plakins, anti-alpha-2-macroglobulin like 1, and anti-desmogleins autoantibodies. The mortality rate of PAMS is up to 50%. This is due on the one hand to the poor response to treatments and on the other hand to the delay in the diagnosis and to the prognosis of the underlying neoplasia.
副肿瘤自身免疫性多器官综合征(PAMS)最初由Anhalt在1990年描述为副肿瘤天疱疮,是一种潜在的致死性水疱疾病,以多形态临床特征为特征,包括皮肤粘膜糜烂、水疱、地衣样丘疹和红斑。PAMS患者血清中检测到多种自身抗体,包括抗血小板抗体、抗α -2-巨球蛋白样1抗体和抗粘粒蛋白自身抗体。PAMS的死亡率高达50%。这一方面是由于对治疗的反应较差,另一方面是由于诊断和潜在肿瘤的预后延迟。
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引用次数: 3
Cutaneous tricholemmal carcinoma: a 15-years single centre experience. 皮肤滴管癌:15年的单中心经验。
IF 2 Q3 Medicine Pub Date : 2020-10-16 DOI: 10.23736/S0392-0488.20.06672-9
F. Boggio, E. Guanziroli, Marco Barella, L. Venegoni, E. Berti, S. Ferrero, A. Coggi, R. Gianotti, A. Del Gobbo
BACKGROUNDClear cell morphology has been described in several cutaneous neoplasms either as a specific feature of some entities either as a morphological variant in the spectrum, and these two entities are frequently considered together in the differential diagnosis.METHODSWe reviewed our series of cases occurred in our laboratory in order to further quantify the number of cases showing morphological features of tricholemmal differentiation and to investigate other clinical or histological difference. We retrieved 91 cases and, for each of them, all the clinical data regarding age, sex, clinical features, and clinical suspicious were collected, when available.RESULTSThe revision of the specimens concluded with a final diagnosis of tricholemmal carcinoma in 15 cases (17%), all the other cases were thus considered as squamous cell carcinoma with clear cell features. No statistically significant correlations were observed with the demografic or clinicopatholagical parameters such as age, sex or dimensions, but morphological revision highlighted a potentially greater "vertical" growth frequently not matched by a concomitant radial one in tricholemmal carcinoma than in squamous tumors.CONCLUSIONSThe debate upon the diagnostic distinction of these tumours is still ongoing with authors proposing the tricholemmal carcinoma as a variant of a squamous cell carcinoma rather than a distinct entity. Further studies are needed to confirm our data and to evaluate the reproducibility of this feature.
背景:在几种皮肤肿瘤中,透明细胞形态被描述为某些实体的特定特征,或者是光谱中的形态变异,这两种实体经常被认为是鉴别诊断的共同因素。方法回顾本实验室发生的一系列病例,以进一步量化表现出滴管分化形态特征的病例数量,并探讨其他临床或组织学差异。我们检索了91例病例,并收集了每个病例的所有临床资料,包括年龄、性别、临床特征和临床可疑性。结果15例(17%)经标本修订最终诊断为滴管癌,其余均为具有透明细胞特征的鳞状细胞癌。与人口统计学或临床病理参数(如年龄、性别或体型)没有统计学上的显著相关性,但形态学修正强调,与鳞状肿瘤相比,口孔癌的潜在“垂直”生长往往与伴随的放射状生长不匹配。结论:关于这些肿瘤的诊断区别的争论仍在进行中,作者提出口孔癌是鳞状细胞癌的一种变体,而不是一个独特的实体。需要进一步的研究来证实我们的数据,并评估这一特征的可重复性。
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引用次数: 0
Frequency of allergic contact dermatitis in hidradenitis suppurativa patients. 化脓性汗腺炎患者过敏性接触性皮炎的发生率。
IF 2 Q3 Medicine Pub Date : 2020-10-16 DOI: 10.23736/S0392-0488.20.06660-2
C. Patruno, G. Fabbrocini, M. D'andrea, S. Dastoli, C. Marasca, M. Napolitano
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引用次数: 1
Urticarial rash in autoinflammatory syndromes: a report of three cases. 自体炎症综合征并发荨麻疹:附3例报告。
IF 2 Q3 Medicine Pub Date : 2020-10-16 DOI: 10.23736/S0392-0488.20.06671-7
P. Facheris, A. D’Arino, G. Pavia, M. Valenti, G. Guidelli, R. Borroni, A. Costanzo, A. Narcisi
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引用次数: 0
A retrospective study on clinical subtypes and management of morphea in 10 Italian Dermatological Units. 意大利10个皮肤科单位morphea临床亚型及治疗回顾性研究。
IF 2 Q3 Medicine Pub Date : 2020-10-16 DOI: 10.23736/S0392-0488.20.06632-8
A. Virdi, A. Patrizi, S. Cambiaghi, A. Diociaiuti, M. El Hachem, D. Schena, A. Bassi, D. Bonamonte, V. Brazzelli, A. Belloni Fortina, P. Pepe, V. Di Lernia, I. Neri
BACKGROUNDThere are still few dermatological studies on morphea. We evaluated the epidemiological and clinical features and management of pediatric morphea, reporting dermatologists experience.METHODSA multicentre retrospective observational study was carried out on the epidemiological and clinical features and management of the disease between 01/01/2009 and 01/10/2014 in 10 Italian Dermatological Units.RESULTSWe collected the data of 69 children affected by: circumscribed morphea (39.1%); linear morphea of trunk and limbs (14.5%); en coupe de sabre morphea (ECDS) (14.5%); progressive facial hemiatrophy (8.7%); generalized form (18.8%); mixed morphea (4.4%). The mean age at onset was 6.86±3.21 years, mainly between 2 and 8 years, but is statistically significantly lower for ECDS (4.5±3.03). Localizations were: head/neck (30.4%), limbs (26.1%), trunk (14.5%), 2 or more sites (29%), most often the trunk plus limbs. Extracutaneous manifestations were observed in 26.1% patients. 10 patients presented a second autoimmune disorder. Treatments were topical in 26.1% cases and systemic (alone or associated with topical treatments) in 68.1%.CONCLUSIONSThere was a lack of uniformity in the management of patients and an increasing awareness of dermatologists on the use of systemic therapies, in particular of methotrexate, which is no longer exclusive to rheumatologists. Methotrexate causes stabilization and improvement of the clinical signs, but topical creams are still considered adjuvant or maintenance therapies during/after the use of systemic drugs.
关于吗啡的皮肤病学研究仍然很少。我们评估了流行病学和临床特征以及儿科吗啡的处理,报告了皮肤科医生的经验。方法对意大利10家皮肤科2009年1月1日至2014年10月1日收治的该病流行病学、临床特征及治疗进行多中心回顾性观察研究。结果我们收集了69例儿童的数据:局限性吗啡中毒(39.1%);躯干和四肢线形分布(14.5%);剑齿炎双发(ECDS) (14.5%);进行性面部偏瘫(8.7%);广义型(18.8%);混合性吗啡(4.4%)。平均发病年龄为6.86±3.21岁,主要在2 ~ 8岁之间,但ECDS的平均发病年龄(4.5±3.03)有统计学意义。定位部位为头颈部(30.4%)、四肢(26.1%)、躯干(14.5%)、2个及以上(29%),以躯干加四肢多见。26.1%的患者有皮肤外表现。10例患者出现第二种自身免疫性疾病。局部治疗占26.1%,全身(单独或联合局部治疗)占68.1%。结论:患者管理缺乏统一性,皮肤科医生对使用全身治疗的认识日益提高,特别是甲氨蝶呤,这不再是风湿病医生的专属。甲氨蝶呤可以稳定和改善临床症状,但局部乳霜仍被认为是全身用药期间/之后的辅助或维持治疗。
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引用次数: 1
Vegas xanthoma: a challenging diagnosis in dermatology. 维加斯黄色瘤:皮肤科的一个具有挑战性的诊断。
IF 2 Q3 Medicine Pub Date : 2020-10-16 DOI: 10.23736/S0392-0488.20.06649-3
V. Gaspari, Rossella Lacava, A. Barisani, C. Misciali, A. D’antuono, B. Raone, S. Vaccari
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引用次数: 0
Methotrexate-induced toxic epidermal necrolysis in a child undergoing a combination therapy for systemic lupus erythematosus: a rare case report. 甲氨蝶呤诱导的中毒性表皮坏死松解在接受系统性红斑狼疮联合治疗的儿童:一个罕见的病例报告。
IF 2 Q3 Medicine Pub Date : 2020-10-09 DOI: 10.23736/S0392-0488.20.06621-3
Huotao Li, Jiao Zhang, Yongfeng Chen
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引用次数: 0
Efficacy of Certolizumab Pegol in naïve versus multi-treated patients affected by psoriatic arthritis. Certolizumab Pegol治疗银屑病关节炎naïve与多次治疗患者的疗效。
IF 2 Q3 Medicine Pub Date : 2020-10-09 DOI: 10.23736/S0392-0488.20.06623-7
A. Dattola, L. Vollono, M. V. Cannizzaro, R. D. Caposiena Caro, S. Mazzilli, G. Melino, E. Candi, E. Campione, L. Bianchi
BACKGROUNDThe efficacy and safety of Certolizumab Pegol over 52 weeks was compared in two groups of patients: Group 1 comprised patients naïve to biologic treatments; Group 2 comprised patients previously treated with one or more anti-Tumor necrosis factor (TNF)-alpha and/or antiinterleukin (IL) agents.METHODSWe reported results in 50 patients affected by both mild psoriasis (PsO) and psoriatic arthritis (PsA). Primary endpoint was a reduction from baseline at week 52 of Disease Activity Score (DAS44-ESR) in both groups of patients. Secondary endpoints were a reduction from baseline at week 52 of Psoriasis Area Severity Index (PASI), Visual Analog Scale for Pain (PAIN VAS), ESR, CRP, and Dermatology life quality index (DLQI).RESULTSWe observed a statistically significant improvement of both cutaneous and rheumatic disease in all patients, with a consistent reduction of DAS44-ESR, PASI, and PAIN VAS from baseline to week 52. DAS44-ESR decreased from 3.9 at BL to 1.5 at W52 (Group 1), and from 3.8 to 1.7 at W52 (Group 2). Mean PASI score decreased from 3.2 at baseline (BL) to 0.4 at W52 (Group 1), and from 5.4 to 0.7 at W52 (Group 2). Mean PAIN-VAS decreased from a value of 73.5 at BL to 2.5 at W52 (Group 1), and from a value of 62.4 at BL to 9.2 at W52 (Group 2). We also found a reduction in ESR, CRP and DLQI values for each time point.CONCLUSIONSOur results confirm that CZP can be administered safely and effectively to treat both psoriasis and psoriatic arthritis irrespective of previous treatments with biologic agents.
背景:在两组患者中比较了Certolizumab Pegol在52周内的疗效和安全性:第一组患者naïve接受生物治疗;第2组包括先前接受过一种或多种抗肿瘤坏死因子(TNF)- α和/或抗白细胞介素(IL)药物治疗的患者。方法我们报告了50例轻度银屑病(PsO)和银屑病关节炎(PsA)患者的结果。主要终点是两组患者在第52周的疾病活动评分(DAS44-ESR)较基线降低。次要终点是第52周时银屑病区域严重程度指数(PASI)、疼痛视觉模拟量表(Pain VAS)、ESR、CRP和皮肤病生活质量指数(DLQI)较基线的降低。结果:我们观察到所有患者的皮肤和风湿病均有统计学上的显著改善,DAS44-ESR、PASI和PAIN VAS从基线到第52周持续下降。DAS44-ESR从3.9下降1.5在提单W52(组1),并在W52从3.8到1.7(组2)。意思是PASI评分从基线值3.2降低到了0.4(提单)W52(组1),并在W52从5.4到0.7(组2)。意思是PAIN-VAS减少从提单值为73.5到2.5在W52(组1),在提单和值为62.4到9.2在W52(组2)。我们还发现减少ESR、CRP和DLQI值为每个时间点。结论与以往的生物制剂治疗相比,CZP可安全有效地治疗银屑病和银屑病关节炎。
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引用次数: 0
Atypical pemphigus: autoimmunity against desmocollins and other non-desmoglein autoantigens. 非典型天疱疮:对粘连蛋白和其他非粘连蛋白自身抗原的自身免疫。
IF 2 Q3 Medicine Pub Date : 2020-10-09 DOI: 10.23736/S0392-0488.20.06619-5
B. Gualtieri, A. Marzano, S. Grando
In this review, we recap current knowledge about non-desmoglein autoantigens in atypical forms of autoimmune pemphigus. More than 50 keratinocyte proteins, including adhesion molecules, receptors and enzymes as well as mitochondrial proteins can be targeted, leading to alterations in numerous intracellular signaling pathways. Patients with pemphigus herpetiformis feature various combinations of antibodies to desmogleins 1 and 3 and desmocollins 1-3. Pemphigus vulgaris patients who do not have antibodies to desmogleins develop typical clinical and histological features of pemphigus. Experimental results revealed synergy of different autoantibodies. Alterations of the keratinocyte adhesive function caused by a single antibody alone are reversible due to self-repair. Since composition of the pool of the most common pathogenic antibodies appears to be similar among pemphigus patients with or without antidesmoglein antibodies, the atypical pemphigus represents a unique model for elucidation of the molecular mechanisms of autoimmunity against non-desmoglein antigens. Further studies of the immunopathology of atypical pemphigus should shed new lights on the pathophysiology of conventional variants of autoimmune pemphigus.
在这篇综述中,我们回顾了目前关于非典型自身免疫性天疱疮的非黏着蛋白自身抗原的知识。超过50种角质细胞蛋白,包括粘附分子,受体和酶以及线粒体蛋白可以被靶向,导致许多细胞内信号通路的改变。疱疹样天疱疮患者具有各种粘连蛋白1和3和粘连蛋白1-3抗体的组合。寻常型天疱疮患者谁没有抗体的粘连蛋白发展天疱疮的典型临床和组织学特征。实验结果显示不同的自身抗体有协同作用。单抗体引起的角质细胞粘附功能的改变由于自我修复是可逆的。由于天疱疮患者中最常见的致病抗体池的组成似乎是相似的,因此非典型天疱疮代表了一种独特的模型,用于阐明针对非桥蛋白抗原的自身免疫的分子机制。对非典型天疱疮免疫病理的进一步研究将为自身免疫性天疱疮的常规变异提供新的病理生理学线索。
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引用次数: 7
期刊
Giornale Italiano Di Dermatologia E Venereologia
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