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Simultaneous Development of SCC of the head and BCC of the leg: Successful Surgical Approach in Bulgarian patient 头部鳞状细胞癌和腿部基底细胞癌的同时发展:保加利亚患者成功的手术入路
Pub Date : 2021-04-12 DOI: 10.15226/2378-1726/8/1/001131
Dr Nikhil Oliveira, Prof Dr Georgi Tchernev
Non-melanoma skin cancer (NMSC) is one of the most common cutaneous malignancies, with an ever-increasing incidence rate worldwide [1]. Studies have shown an association between intermittent and chronic sunlight exposure and the development of BCC and SCC respectively [2]. UV radiation is a known carcinogen that can induce mutations in the p53 gene, known also as the guardian of the genome [3]. This can lead to persistent damage and failure to eliminate dysplastic cells as subsequent mechanisms like p21 and p16 activation fail to arrest cell cycle. Proapoptotic proteins such as BAK and BAX are also unable to activate programmed cell death, ultimately leading to the formation of precancerous lesions and NMSC [3, 4].
非黑色素瘤皮肤癌(Non-melanoma skin cancer, NMSC)是最常见的皮肤恶性肿瘤之一,在世界范围内发病率不断上升[1]。研究表明,间歇性和慢性阳光照射分别与BCC和SCC的发生有关[2]。紫外线辐射是一种已知的致癌物质,可以诱导p53基因发生突变,而p53基因又被称为基因组的守护者[3]。由于p21和p16激活等后续机制无法阻止细胞周期,这可能导致持续损伤和无法消除发育不良细胞。BAK和BAX等促凋亡蛋白也无法激活程序性细胞死亡,最终导致癌前病变和NMSC的形成[3,4]。
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引用次数: 0
Bullous Pemphigoid in Children: Clinical Presentation, Pathology and Management 儿童大疱性类天疱疮:临床表现、病理和治疗
Pub Date : 2021-03-29 DOI: 10.15226/2378-1726/8/1/001130
Faris Oumeish, H. Hamad, Hussain Tukmatchy, Niall Scully, M. Al Abadie
Bullous pemphigoid rarely presents in childhood age group and when it presents it has two age peaks infantile and childhood forms. Key presenting features of childhood BP include subepidermal blistering, eosinophilia and severe itching.The histological features of bullous pemphigoid are the same as those described in adulthood and vary with the age of the lesion.Childhood BP tends to have a generally good prognosis with appropriate therapy Keywords: Bullous Pemphigoid; Autoimmune Disorders; Blistering Diseases
大疱性类天疱疮很少出现在儿童年龄组,当它出现时,它有两个年龄高峰,婴儿和儿童形式。儿童BP的主要表现特征包括表皮下起泡、嗜酸性粒细胞增多和严重瘙痒。大疱性类天疱疮的组织学特征与成年期相同,并随病变年龄而变化。关键词:大疱性类天疱疮;自身免疫性疾病;猛烈的疾病
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引用次数: 1
Review: Atopic Dermatitis - Exposome, Microbiome, Psyche,and Evolution 综述:特应性皮炎——暴露体、微生物群、心理和进化
Pub Date : 2021-02-02 DOI: 10.15226/2378-1726/8/1/001129
J. Penev, M. Balabanova, I. Bakardzhiev
Atopic Dermatitis (AD) is probably the most common type of eczema. The quality of life assessment reveals a number of negative consequences for patients with AD and their families. AD is associated with high levels of stress, stigma, social withdrawal, anxiety, depression, and even suicide ideation. Atopic dermatitis is a multisystem inflammatory disease with a multifactorial, and still controversial etiology. Complex interactions between genetic, psychoneuroimmune and endocrine factors, microbiome, exposome and environment, mental and social factors, diet and lifestyle are proposed. AD is difficult to treat, has a chronic course with peaks of exacerbation and pronounced manifestation of mental factors. Such complexity requires deeper understanding of mechanisms behind AD. We consider a possible psychogenic component in diseaseexacerbation, andoffera different point of view onrelationships of human body with the colonizing microbiomein the context of AD etiology and pathogenesis. These relationships are interpreted in evolutionary terms in an attempt to shed some light on mechanisms of chronic and pathological skin processes. Keywords: Atopic Dermatitis; Microbiome; Exposome;Obsessive- Compulsive Disorder
特应性皮炎(AD)可能是最常见的湿疹类型。生活质量评估揭示了AD患者及其家庭的一些负面影响。阿尔茨海默病与高水平的压力、耻辱、社交退缩、焦虑、抑郁甚至自杀念头有关。特应性皮炎是一种多因素的多系统炎症性疾病,其病因仍有争议。遗传因素、心理神经免疫因素和内分泌因素、微生物组、暴露物和环境因素、心理和社会因素、饮食和生活方式之间存在复杂的相互作用。阿尔茨海默病难以治疗,病程较长,有加重高峰,精神因素表现明显。这种复杂性需要对AD背后的机制有更深的理解。我们考虑了疾病恶化的可能的心理因素,并在阿尔茨海默病的病因和发病机制的背景下对人体与定植微生物群的关系提出了不同的观点。这些关系被解释在进化方面,试图揭示一些机制的慢性和病理性的皮肤过程。关键词:特应性皮炎;微生物;暴露;强迫症
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引用次数: 0
The Simultaneous Inhibition of IL‑4 and IL‑13 by Dupilumab Dupilumab对IL - 4和IL - 13的同时抑制作用
Pub Date : 2021-01-01 DOI: 10.15226/2378-1726/8/2/001138
Ana Paula Galli Sanchez, Tatiane Ester Aidar Fernandes
Interleukins (IL) IL-4 and IL-13 are key players in diseases in which the Type 2 immune response is predominant, such as atopic dermatitis (AD), asthma, and chronic rhinosinusitis with nasal polyposis (CRSwNP), that are currently being treated with dupilumab. Dupilumab is a fully human IgG4 monoclonal antibody that targets the IL-4 receptor alpha chain (IL-4Rα), preventing both IL-4 and IL-13 mediated signaling. This mini-review summarizes the IL-4 receptor system as well as the mechanism of action of dupilumab. Keywords: IL-4; IL-13; dupilumab; Type 2 immunity Abbreviations AD: Atopic Dermatitis CRSwNP: Chronic Rhinosinusitis with Nasal Polyps DNA: Deoxyribonucleic Acid JAK: Janus Kinase IL: Interleukin IL-4R: Interleukin 4 Receptor IL-4Rα: Interleukin 4 Receptor alpha chain IL-13Rα1: Interleukin 13 Receptor alpha 1 chain IL-13Rα2:Interleukin 13 Receptor alpha 2 chain STAT: Signal Transducer and Activator of Transcription TGF-β: Transforming Growth Factor beta TNF: Tumor Necrosis Factor TYK2: Tyrosine Kinase 2 γc: Common gamma chain
白细胞介素(IL) IL-4和IL-13在2型免疫应答占主导地位的疾病中发挥着关键作用,如特应性皮炎(AD)、哮喘和慢性鼻窦炎伴鼻息肉病(CRSwNP),这些疾病目前正在用dupilumab治疗。Dupilumab是一种全人源IgG4单克隆抗体,靶向IL-4受体α链(IL-4Rα),阻止IL-4和IL-13介导的信号传导。本文就IL-4受体系统及dupilumab的作用机制进行综述。关键词:il - 4;IL-13;dupilumab;2型免疫简写AD:特应性皮炎CRSwNP:慢性鼻窦炎伴鼻息肉DNA:脱氧核糖核酸JAK: Janus激酶IL:白介素IL- 4r:白介素4受体IL- 4r α:白介素4受体α链IL- 13r α1:白介素13受体α1链IL- 13r α2:白介素13受体α2链STAT:转录信号转导和激活因子TGF-β:转化生长因子β TNF:肿瘤坏死因子TYK2:酪氨酸激酶2 γc:共同γ链
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引用次数: 0
Borderline Intermediate Thickness Cutaneous Melanoma Class B: Isn’t it Time for Personalised One Step Surgical Approach as Standard Clinical Behaviour? 边缘性中等厚度皮肤黑色素瘤B级:个体化一步手术方法是否应该成为标准临床行为?
Pub Date : 2021-01-01 DOI: 10.15226/2378-1726/8/2/001137
Kandathil Lj, O. N, Patterson Jw, Tchernev G
We present a 40-year-old female who visited our clinic with a solitary lesion on the posterolateral aspect of the lower left leg (Figure 1a). She noticed a progressive change in the size and shape of the lesion and decided to consult a dermatologist in March 2021. The patient had a history of Hashimoto’s thyroiditis that was well-controlled onlevothyroxine. No other comorbidities were reported and she was otherwise healthy. During the clinical examination a single pigmented patch measuring 2.5 cm in greatest diameter was identified. Morphologically the lesion was asymmetrical with irregular borders and uneven colour. At the centre, an exudative, ulcerated nodule was also noted (Figure1a-f). Clinical and dermatoscopic findings were consistent with the diagnosis of a superficial spreading cutaneous melanoma. Ultrasound diagnostics of the abdominal cavity and retroperitoneal organs showed no signs of tumor spread. Chest radiography was also within normal limits. Laboratory testing showed an elevated uric acid level of 456 μmol/l (reference range 142 - 340 μmol/l), but otherwise all other parameters were normal. Following the recommended American Joint Committee on Cancer (AJCC) guidelines, we performed a primary resection with 0.5 cm margins in all directions. The resected tissue was subsequently sent for histopathological evaluation and confirmed the diagnosis of borderline intermediate thickness malignant melanoma - class B, 4 mm Breslow thickness, Clark IV, (pT4BN0M0) (Figure2a-d). There was high mitotic activity but no spontaneous regression, insignificant lymphocytic stromal reaction and clear resection margins. Post diagnostic workup, including chest and abdominal CT, showed no signs of metastatic dissemination. One week later, the patient was sent to the National Oncology Hospital for re-excision and Sentinel Lymph Node Biopsy (SLNB). The re-excision of additional 2cm from the previous surgical scar was conducted in parallel with removal of the draining sentinel lymph node (Figures 3a & 3d). The closure of the defect after re-excision led to the unfortunate complication of wound dehiscence and failure to close successfully (Figure 3b-c). However, after several sessions of debridement, cleansing, rebandaging and administration of antibiotics, there was visible improvement with subsequent resolution(Figure 3d-f). Since that time, she has been in excellent condition, and no complications have been reported to date.
我们报告了一位40岁的女性,她就诊于我们的诊所,左下肢后外侧有一个孤立的病变(图1a)。她注意到病变的大小和形状逐渐发生变化,并决定在2021年3月咨询皮肤科医生。患者有桥本甲状腺炎病史,经左旋甲状腺素控制良好。没有其他合并症的报道,她是健康的。在临床检查中发现了一个最大直径2.5 cm的单个色素斑。形态学上病灶不对称,边界不规则,颜色不均匀。中心可见一渗出性溃疡性结节(图1a-f)。临床和皮肤镜检查结果与浅表扩散皮肤黑色素瘤的诊断一致。超声诊断腹腔及腹膜后脏器未见肿瘤扩散迹象。胸部x线检查也在正常范围内。实验室检查显示尿酸水平升高456 μmol/l(参考范围142 - 340 μmol/l),但其他各项参数正常。根据美国癌症联合委员会(AJCC)推荐的指南,我们在所有方向上进行了0.5 cm边缘的原发性切除。随后将切除的组织送去进行组织病理学评估,确诊为交界性中厚恶性黑色素瘤- B级,4mm Breslow厚度,Clark IV, (pT4BN0M0)(图2a-d)。有丝分裂活性高,但无自发消退,淋巴细胞间质反应不明显,切除边缘清晰。诊断后检查,包括胸部和腹部CT,未发现转移性传播的迹象。一周后,患者被送往国家肿瘤医院进行再次切除和前哨淋巴结活检(SLNB)。在切除引流前哨淋巴结的同时,再次切除先前手术疤痕的2cm(图3a和3d)。再次切除后缺损的闭合导致了不幸的并发症,即伤口裂开和未能成功闭合(图3b-c)。然而,经过几次清创、清洁、重新包扎和抗生素治疗后,患者的病情得到了明显改善(图3d-f)。从那时起,她的身体状况非常好,到目前为止没有并发症的报道。
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引用次数: 0
The JAK-STAT Pathway and the JAK Inhibitors JAK- stat通路和JAK抑制剂
Pub Date : 2020-11-30 DOI: 10.15226/2378-1726/7/5/001128
Ana Paula Galli Sanchez, Tatiane Ester Aidar Fernandes, Gustavo Martelli Palomino
Dozens of cytokines that bind Type I and Type II receptors use the Janus Kinases (JAK) and the Signal Transducer and Activator of Transcription (STAT) proteins pathway for intracellular signaling, orchestrating hematopoiesis, inducing inflammation, and controlling the immune response. Currently, oral JAK inhibitors are being used to treat many inflammatory and myeloproliferative diseases and are also under investigation in several clinical trials for skin diseases. Thus, dermatologists should understand how the JAK-STAT pathway works as well as the mechanism of action of the JAK inhibitors which will certainly become an important part of the dermatologist’s treatment armamentarium in the next few years. Keywords: JAK inhibitors; Janus Kinases; JAK-STAT Pathway List of Abbreviations: AD: Atopic Dermatitis ADP: Adenosine diphosphate Dmards: Disease-Modifying Antirheumatic Drugs JAK: Janus kinase(s) Jaki: Janus kinase Inhibitor(s) PIAS: Protein Inhibitor of Activated STAT P-STAT: Phosphorylated STAT STAT: Signal Transducer and Activator of Transcription TYK2: Tyrosine Kinase 2 Wsxws: Tryptophan-Serine-X-Tryptophan-Serine
结合I型和II型受体的数十种细胞因子使用Janus激酶(JAK)和信号转导和转录激活因子(STAT)蛋白途径进行细胞内信号传导,协调造血,诱导炎症和控制免疫反应。目前,口服JAK抑制剂正被用于治疗许多炎症性和骨髓增殖性疾病,并且在一些皮肤病的临床试验中也在研究中。因此,皮肤科医生应该了解JAK- stat通路是如何工作的,以及JAK抑制剂的作用机制,这必将成为未来几年皮肤科医生治疗手段的重要组成部分。关键词:JAK抑制剂;Janus激酶;JAK-STAT通路简写列表:AD:特应性皮炎ADP:二磷酸腺苷类药物:疾病改善抗风湿药物JAK: Janus激酶(s) Jaki: Janus激酶抑制剂(s) PIAS:活化STAT蛋白抑制剂P-STAT:磷酸化STAT STAT:信号转导和转录激活因子TYK2:酪氨酸激酶2 Wsxws:色氨酸-丝氨酸- x -色氨酸-丝氨酸
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引用次数: 1
Drug Induced Cancers: Simultaneously Development of Cutaneous Melanoma, Colon Carcinoma and Kaposi Sarcoma under Valsartan/ Hydrochlorothiazide 药物诱导的癌症:缬沙坦/氢氯噻嗪可同时发展皮肤黑色素瘤、结肠癌和卡波西肉瘤
Pub Date : 2020-11-06 DOI: 10.15226/2378-1726/7/5/001127
G. Tchernev, G. Poterov
The side effects of antihypertensive drugs are often the subject of discussions, publications and presentations, but not in cases where they include in their “palette” or “repertoire” concepts such as generating / promoting or potentiating various types of cancer, as well as metastasis of already existing one. Unfortunately, progress within the real, clinical medicine often does not start with the desired results (from those expected within clinical observations or experimental data after administration of a particular substance or drug). Breakthroughs in medicine and progress start with the careful registration and analysis of unwanted and unexpected, unplanned results. Again, unfortunately, the analysis of it is often swept under the table, without thinking that the negative results should be the generator of progress. When the observations of certain clinical or experimental data remain insufficiently analyzed, neglected or deliberately hidden - the result is often alarming or fatal - similar to the scandals (from 2018) with Sartans and the generation of hundreds of cutaneous melanomas, as well as heterogeneous species and other cancerous forms. However, if we all focus together on the problems through the prism of the possibilities to solve them, there is a real chance to achieve a drastic decrease in the incidence of a number of tumor diseases, due to the possibility of rethinking their pathogenesis. The article is a kind of analysis and directing clinicians in the right direction in relation to an urgent need to clarify issues related to the procarcinogenic effect of drugs from the so-called group of Sartans and Thiazide diuretics. In the first mentioned drugs, the carcinogenic effect could be due to: 1) the action of the main substance of the Sartans themselves (proven in experimental conditions, but also in in vivo data) and 2) the additive, undesirable ingredients or the so-called nitrosamines arising as additional contamination within the production process. The combination of Sartans and Thiazide diuretics (plus Nitrosamines, the presence of which is not adequately tested) could in all likelihood have a fatal, mutually potentiating procarcinogenic effect, leading to the manifestation of 3 or even more cancers simultaneously. This publication presents for the first time in the world literature a patient who developed three cancers at the same time (Kaposi’s sarcoma, skin melanoma and colon cancer) after starting therapy with Valsartan and hydrochlorothiazide. Our critical analysis is focused on the regulatory authorities in the face of the FDA, EMA, but also on the National Agency for Drug Control. Attention is also paid to the non-traditional and illegal from the point of view of all ethical norms ways of influencing the pharmaceutical industry, in order to protect its positions in the framework of the daily emerging world scandals. Keywords: Valsartan, Hydrochlorothiazide; Cutaneous Melanoma; Colon Carcinoma; Kaposi Sarcoma; Drug Indu
降压药的副作用经常是讨论、出版物和报告的主题,但在它们的“调色板”或“曲目”概念中不包括诸如产生/促进或加强各种类型的癌症以及已经存在的癌症的转移等概念。不幸的是,实际临床医学的进展往往不是从期望的结果开始的(从临床观察或服用特定物质或药物后的实验数据中所期望的结果开始)。医学的突破和进步始于对不想要的、意外的、计划外的结果的仔细登记和分析。不幸的是,对它的分析常常被隐藏起来,而没有想到消极的结果应该成为进步的动力。当某些临床或实验数据的观察结果仍然没有得到充分分析、被忽视或故意隐藏时,结果往往是令人震惊或致命的——类似于(2018年)沙坦和数百种皮肤黑色素瘤的产生,以及异质物种和其他癌症形式的丑闻。然而,如果我们都通过可能性的棱镜来关注这些问题来解决它们,由于重新思考其发病机制的可能性,有一个真正的机会来实现一些肿瘤疾病发病率的急剧下降。这篇文章是一种分析和指导临床医生正确的方向,涉及迫切需要澄清与所谓的沙坦类和噻嗪类利尿剂组药物的前致癌作用有关的问题。在第一个提到的药物中,致癌作用可能是由于:1)sartan本身的主要物质的作用(在实验条件下得到证实,但在体内数据中也得到证实)和2)添加剂、不良成分或所谓的亚硝胺在生产过程中作为额外污染产生。沙坦类药物和噻嗪类利尿剂(加上亚硝胺类药物,其存在程度尚未得到充分测试)的联合使用很可能具有致命的、相互增强的前致癌作用,导致3种甚至更多癌症同时出现。本出版物在世界文献中首次报道了一名患者在开始使用缬沙坦和氢氯噻嗪治疗后同时患上三种癌症(卡波西肉瘤、皮肤黑色素瘤和结肠癌)。我们的批判性分析主要集中在面对FDA, EMA的监管机构,以及国家药物管制局。从所有道德规范的角度来看,还注意影响制药业的非传统和非法方式,以保护其在每天出现的世界丑闻框架中的地位。关键词:缬沙坦;氢氯噻嗪;皮肤的黑色素瘤;结肠癌癌;卡波济肉瘤;药物致癌;食品及药物管理局;诺华;教育津贴
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引用次数: 4
Successful Treatment of Pemphigus Vulgaris with a Modified Regimen of Rituximab Biosimilar(CT-P10) Combined with IVIG 改良Rituximab生物类似药(CT-P10)联合IVIG成功治疗寻常型天疱疮
Pub Date : 2020-11-02 DOI: 10.15226/2378-1726/7/5/001126
W. I. Kim, K. Hong, Sooyoung Kim, Moon Kyun Cho, K. Whang, Hyun-Sook Kim
Keywords: Pemphigus vulgaris;Modified Regimen;Rituximab biosimilar; IVIG Pemphigus Vulgaris (PV) has been known as fatal autoimmune bullous diseases affecting the skin and mucous membranes. Here, we report that a patient with PV responded rapidly to a modified regimen of rituximab biosimilar (CT-10, Truxima®)combined with Intravenous Immunoglobulin (IVIG). A 53-year-old man presented with extensive erosions due to blistering on the whole body following initial mucosal erosions which started at 3 years ago. Laboratory tests revealed an eosinophil count of 16.7% (normal range: 0~5%) with normal liver and renal functions. Antinuclear antibody was a nuclear membrane pattern with a titer of 1:40. Anti-dsDNA, anti-Ro/La, anti-Scl-70, and anti-phospholipid antibodies were negative. Myeloperoxidase and PR3 antineutrophil cytoplasmic autoantibody were both negative. Histological examination showed suprabasala cantholysis, while direct and indirect immunofluorescence assays demonstrated a deposition of immunoglobulin G (IgG) on anintercellular surface at 1:640 dilutions (Figure. 1a, b). Based on the blistering on the whole body and characteristic biopsy result, the patient was diagnosed as PV. Although he was initially treated with topical and highdose methylprednisolone (1000 mg/day) pulse therapy for 3 consecutive days, there was no significant improvement in skin blistering lesions. We decided to administer CT-P10 combined with IVIG. We treated the patient with CT-P10 (500, 500, and 1000mg once weekly for 3 weeks) combined with IVIG (1g/ kg at first and third week). During the treatment of CT-P10 combined with IVIG, a clinical response rapidly appeared, and levels of antibodies were decreased from 1:640 to 1:40 (Figure. 1c). Disease activity was dramatically controlled within 3 weeks. It achieved the end of the consolidation phase for PV within 5 weeks after starting CT-P10 combined with IVIG (Figure. 1dg). Then, we tapered oral prednisolone and steroid-sparing agents including mycophenolate mofetil and cyclosporine for maintenance therapy. Until now, there has been no recurrence of PV on 36 months follow up.
关键词:寻常型天疱疮;改良方案;利妥昔单抗生物仿制药;寻常型天疱疮(Pemphigus Vulgaris, PV)是一种影响皮肤和粘膜的致死性自身免疫性大疱性疾病。在这里,我们报告了一名PV患者对改良的利妥昔单抗生物类似药(CT-10, Truxima®)联合静脉注射免疫球蛋白(IVIG)的方案迅速反应。53岁男性,3年前开始出现最初的粘膜糜烂,由于全身起泡而出现大面积糜烂。实验室检查显示嗜酸性粒细胞计数16.7%(正常范围:0~5%),肝肾功能正常。抗核抗体为核膜型,滴度为1:40。抗dsdna、抗ro /La、抗scl -70和抗磷脂抗体均为阴性。髓过氧化物酶和抗中性粒细胞胞浆自身抗体PR3均为阴性。组织学检查显示基底上淋巴管溶解,而直接和间接免疫荧光检测显示1:640稀释的免疫球蛋白G (IgG)在细胞间表面沉积(图1a, b)。根据全身起泡和特征性活检结果,诊断为PV。虽然患者最初接受了连续3天的局部和大剂量甲基强的松龙(1000 mg/天)脉冲治疗,但皮肤起泡病变没有明显改善。我们决定使用CT-P10联合IVIG。我们给予CT-P10(500、500和1000mg,每周1次,共3周)联合IVIG (1g/ kg,第1周和第3周)治疗。在CT-P10联合IVIG治疗期间,临床反应迅速出现,抗体水平从1:640降至1:40(图1c)。疾病活动在3周内得到显著控制。CT-P10联合IVIG治疗后5周内PV巩固期结束(图1dg)。然后,我们逐渐减少口服强的松龙和类固醇保留剂,包括霉酚酸酯和环孢素用于维持治疗。到目前为止,随访36个月未见PV复发。
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引用次数: 0
Estrogen Receptor and Progesterone Receptor Positive Pigmented Extramammary Paget’s Disease of the Axilla Mimicking Melanoma: A Case Report and Review of the Literature 雌激素受体和孕激素受体阳性的腋窝样黑色素瘤色素样乳腺外佩吉特病1例报告及文献复习
Pub Date : 2020-10-15 DOI: 10.15226/2378-1726/7/4/001122
Chiyun Wang, H. Chen, Shih-Hao Liu
Pigmented extramammary Paget’s disease (EMPD) is a rare variant which is often confused clinically and histologically with melanoma or other pigmented lesions. Herein, we describe a rare case of pigmented EMPD involving the axilla of a 40-year-old female thought initially to represent malignant melanoma clinically. Immunohistochemically, the neoplastic cells are positive for Estrogen Receptor (ER), Progesterone Receptor (PR), and weakly positive for Human Epidermal Growth Factor Receptor 2 (Her2). A review of the literature reveals that the pigmented variant of EMPD could be easily misdiagnosed as melanoma initially and additional confirmatory studies are almost always needed to confirm the diagnosis. The expression of ER and PR in pigmented EMPD is rarely mentioned previously. We believe it should be further investigated for the understanding of pathogenesis in pigmented EMPD and the potential role of adjuvant hormonal therapy. Keywords: Extramammary Paget’s Disease; Axilla; Estrogen Receptor; Progesterone Receptor
色素性乳腺外佩吉特病(EMPD)是一种罕见的疾病,临床上和组织学上常与黑色素瘤或其他色素性病变混淆。在此,我们描述了一个罕见的病例色素EMPD涉及一个40岁的女性腋窝最初认为代表恶性黑色素瘤临床。免疫组化结果显示,肿瘤细胞雌激素受体(ER)、孕激素受体(PR)阳性,人表皮生长因子受体2 (Her2)弱阳性。文献回顾表明,色素变异的EMPD很容易被误诊为黑色素瘤,并且几乎总是需要额外的确证研究来确认诊断。ER和PR在色素型EMPD中的表达以往很少被提及。我们认为应该进一步研究色素性EMPD的发病机制和辅助激素治疗的潜在作用。关键词:乳腺外佩吉特病;腋下;雌激素受体;孕激素受体
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引用次数: 0
Pseudoporyphyria Associated with Lemon Water and Naproxen 假性卟啉症与柠檬水和萘普生有关
Pub Date : 2020-06-18 DOI: 10.15226/2378-1726/7/2/001115
Nwanneka Okwundu, John Moesch, Sarah Belden
Background: Pseudoporyphyria is an uncommon bullous dermatosis. It shares clinical and histological features with porphyria cutanea tarda, but it occurs in the absence of porphyrin metabolic dysfunction. It is characterized by skin fragility, bullae, milia, and scarring on the dorsum of the hands and other sun-exposed areas. Case: We present a patient on naproxen with recurrent pseudoporyphyria of the dorsal hands associated with the consumption of lemon water. Findings: Biopsy showed a pauci-inflammatory sub epidermal vesicle with caterpillar bodies and laboratory studies lacked any urine or serum porphyrin abnormalities. The cutaneous lesions were noted to resolve with discontinuation of the consumption of lemon water. Keywords: Pseudoporyphyria; Porphyrin; Porphyria Cutanea Tarda; Chlorophyll; Vesicle and Depigmented
背景:假性卟啉症是一种罕见的大疱性皮肤病。它与迟发性皮肤卟啉症具有相同的临床和组织学特征,但在没有卟啉代谢功能障碍的情况下发生。它的特点是皮肤脆弱,大疱,粟粒,手背和其他暴露在阳光下的地方有疤痕。病例:我们提出一个病人的萘普生与反复假性卟啉症的手背与柠檬水的消费有关。结果:活组织检查显示表皮下炎性囊泡伴毛虫体,实验室检查未见尿或血清卟啉异常。皮肤病变被注意到解决与停止饮用柠檬水。关键词:Pseudoporyphyria;卟啉;迟发性皮肤卟啉症;叶绿素;囊泡和脱色
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Journal of Clinical Research in Dermatology
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