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Blastic plasmacytoid dendritic cell neoplasm: a short review and update. 大疱性浆细胞树突状细胞瘤:简短回顾与最新进展。
IF 2.3 Q2 DERMATOLOGY Pub Date : 2023-08-11 eCollection Date: 2024-05-07 DOI: 10.4081/dr.2023.9781
Cesare Massone, Giulia Rivoli, Simona Sola, Emanuele Angelucci

Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare hematologic neoplasm (less than 1% of primary cutaneous lymphomas and acute leukemia) with a highly aggressive clinical course and frequent skin, bone marrow and central nervous system involvement. Even though there is often an early response to chemotherapy, leukemic dissemination relapses are very common and result in poor outcomes, with a median overall survival of 8 to 14 months in the first-line setting using standard combination chemotherapy regimens. Almost 90% of patients experience skin involvement as their initial site of infection, where BPDCN may stay restricted for weeks or even months until a swift secondary phase involving multiple organs takes place. Consequently, it is crucial to suspect and identify early skin lesions, as well as to conduct and report a skin biopsy as soon as possible. In order to diagnose and treat BPDCN, a multidisciplinary strategy involving collaboration between pathologists, hematologists, and dermatologists is unquestionably essential.

增生性浆细胞树突状细胞肿瘤(BPDCN)是一种罕见的血液肿瘤(不到原发性皮肤淋巴瘤和急性白血病的1%),临床病程极具侵袭性,常累及皮肤、骨髓和中枢神经系统。尽管化疗通常会产生早期反应,但白血病扩散复发非常常见,且预后不佳,在一线使用标准联合化疗方案时,中位总生存期为 8 至 14 个月。近 90% 的患者最初的感染部位是皮肤,BPDCN 可能会在皮肤上局限数周甚至数月,直至迅速进入累及多个器官的继发阶段。因此,怀疑和识别早期皮肤病变并尽快进行皮肤活检和报告至关重要。为了诊断和治疗 BPDCN,病理学家、血液学家和皮肤科医生之间合作的多学科策略无疑是至关重要的。
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引用次数: 0
Blastic plasmacytoid dendritic cell neoplasm: a short review and update 母浆细胞样树突状细胞肿瘤:综述与最新进展
Q2 DERMATOLOGY Pub Date : 2023-08-11 DOI: 10.4081/dr.2021.9781
Cesare Massone, Giulia Rivoli, Simona Sola, Emanuele Angelucci
Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare hematologic neoplasm (less than 1% of primary cutaneous lymphomas and acute leukemia) with a highly aggressive clinical course and frequent skin, bone marrow and central nervous system (CNS) involvement. Despite a frequent initial response to chemotherapy, relapses with eventual leukemic dissemination are extremely common, leading to poor outcomes and a median overall survival (OS) ranging from 8 to 14 months in first line setting, with standard combination chemotherapy regimens. The skin is the first affected site (in almost 90% of patients) where BPDCN may remain confined for weeks or even months (sanctuary?) until a rapid second step with multiorgan involvement occurs. Therefore, it is of uppermost importance to suspect and recognize early skin lesions and to perform and report a skin biopsy as soon as possible. A multidisciplinary approach with coordination among dermatologists, pathologists and hematologists is definitively crucial in diagnosis and management of BPDCN.
母浆细胞样树突状细胞肿瘤(BPDCN)是一种罕见的血液肿瘤(不到1%的原发性皮肤淋巴瘤和急性白血病),具有高度侵袭性的临床病程,经常累及皮肤、骨髓和中枢神经系统(CNS)。尽管对化疗的初始反应频繁,但复发并最终白血病传播是极其常见的,导致预后不良,在一线设置中,标准联合化疗方案的中位总生存期(OS)为8至14个月。皮肤是第一个受影响的部位(在几乎90%的患者中),BPDCN可能会持续数周甚至数月(避难所?),直到迅速发生第二步并累及多器官。因此,最重要的是怀疑和识别早期皮肤病变,并尽快进行和报告皮肤活检。在皮肤科医生、病理学家和血液学家的协调下,多学科方法对BPDCN的诊断和管理至关重要。
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引用次数: 0
Langerhans cell histiocytosis presenting as a blueberry muffin rash 朗格汉斯细胞组织细胞增多症表现为蓝莓松饼疹
Q2 DERMATOLOGY Pub Date : 2023-08-09 DOI: 10.4081/dr.2023.9791
Shahad F. Alanazi, Yara Jazzar, Tala Beidas, Haya Soufan, Khaled A. Mohajer, Abdulmalik Alhammad
Langerhans cells, often referred to as the “macrophages of the skin”, are dendritic cells that normally reside in the epidermis and papillary dermis. Just like macrophages, they function as antigen-presenting cells that activate naive T cells. Certain mutations such as those involving the BRAF gene can cause unopposed production of Langerhans Cells, which is known as Langerhans Cell Histiocytosis (LCH). LCH triggers an inflammatory immune response that causes systemic manifestations such as fever and fatigue, as well as other manifestations depending on the affected organs. The pathogenesis behind LCH remains poorly understood. It is still unknown whether it is a neoplastic process or a reactive cancer-mimicking illness. Diagnosis of LCH is confirmed by biopsy, and treatment is largely dependent on the extent and severity of the disease. Common treatments include corticosteroids, excision, radiation, and chemotherapy. We present a case of a 1-year-old Saudi male with LCH.
朗格汉斯细胞,通常被称为“皮肤巨噬细胞”,是一种树突状细胞,通常存在于表皮和乳头状真皮中。就像巨噬细胞一样,它们的功能是抗原呈递细胞,激活幼稚T细胞。某些突变,如涉及BRAF基因的突变,可导致郎格汉斯细胞的无对抗产生,这被称为郎格汉斯细胞组织细胞增多症(LCH)。LCH引发炎症免疫反应,引起全身表现,如发烧和疲劳,以及取决于受影响器官的其他表现。LCH背后的发病机制仍然知之甚少。目前尚不清楚这是一种肿瘤过程还是一种反应性癌症模拟疾病。LCH的诊断是通过活检确认的,治疗在很大程度上取决于疾病的程度和严重程度。常见的治疗方法包括皮质类固醇、切除、放疗和化疗。我们报告一例1岁的沙特男性LCH。
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引用次数: 0
Combination of cyclosporine A and methylprednisolone to treat pediatric Stevens-Johnson syndrome/toxic epidermal necrolysis overlap syndrome. 联合使用环孢素 A 和甲基强的松龙治疗小儿史蒂文斯-约翰逊综合征/毒性表皮坏死重叠综合征。
IF 2.3 Q2 DERMATOLOGY Pub Date : 2023-06-19 eCollection Date: 2023-06-07 DOI: 10.4081/dr.2023.9656
Shinta Trilaksmi Dewi, Laily Noor Qomariah, Widya Khairunisa Sarkowi, Monika Puspitasari, Miya Khalidah, Marcella Anggatama, Dwinanda Almira Rizkiani, Kristiana Etnawati, Sri Awalia Febriana

The treatment of epidermal necrolysis in pediatric patients remains a major challenge. Cyclosporine A has emerged as a promising therapy for epidermal necrolysis in adults; however, its efficacy in children is unclear. We present the case of a boy with Stevens-Johnson syndrome/toxic epidermal necrolysis overlap syndrome who was initially resistant to methylprednisolone monotherapy but improved after receiving the combination of cyclosporine A and methylprednisolone. Published reports on the use of cyclosporine A for pediatric epidermal necrolysis are also briefly reviewed.

治疗儿童表皮坏死症仍是一项重大挑战。环孢素 A 已成为治疗成人表皮坏死症的有效药物,但其对儿童的疗效尚不明确。我们介绍了一例患有史蒂文斯-约翰逊综合征/毒性表皮坏死症重叠综合征的男孩,他最初对甲基强的松龙单药治疗产生耐药性,但在接受环孢素 A 和甲基强的松龙联合治疗后病情有所好转。本文还简要回顾了已发表的关于使用环孢素 A 治疗小儿表皮坏死症的报告。
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引用次数: 0
Environmental factors in autoimmune bullous diseases with a focus on seasonality: new insights. 关注季节性自身免疫性大疱性疾病的环境因素:新见解。
IF 1.1 Q2 DERMATOLOGY Pub Date : 2023-06-14 eCollection Date: 2023-09-12 DOI: 10.4081/dr.2023.9641
Roberto D'Astolto, Lavinia Quintarelli, Alberto Corrà, Marzia Caproni, Luca Fania, Giovanni Di Zenzo, Biagio Didona, Giulia Gasparini, Emanuele Cozzani, Claudio Feliciani

Autoimmune bullous diseases are a heterogeneous group of rare conditions clinically characterized by the presence of blisters and/or erosions on the skin and the mucous membranes. Practically, they can be divided into two large groups: the pemphigoid group and the pemphigus group, depending on the depth of the autoimmune process on the skin. A family history of autoimmune diseases can often be found, demonstrating that genetic predisposition is crucial for their development. Moreover, numerous environmental risk factors, such as solar radiation, drugs, and infections, are known. This study aimed to evaluate how seasonality can affect the trend of bullous pemphigoid and pemphigus vulgaris, especially considering the number of hospitalizations recorded over the course of individual months. The total number of hospitalizations in the twelve months of the year was evaluated. Moreover, blood chemistry assay and, for some patients, enzyme-linked immunosorbent assay were executed to evaluate antibodies. Regarding the severity of the disease, the bullous pemphigoid area index and the pemphigus disease area index score systems were used. Results showed a complex interplay between environmental factors such as seasons and autoimmune conditions.

自身免疫性大疱性疾病是一组罕见的异质性疾病,临床特征是皮肤和粘膜上存在水泡和/或侵蚀。实际上,根据皮肤自身免疫过程的深度,他们可以分为两大组:类天疱疮组和天疱疮。经常可以发现自身免疫性疾病的家族史,这表明遗传易感性对其发展至关重要。此外,许多环境风险因素,如太阳辐射、药物和感染,都是已知的。本研究旨在评估季节性如何影响大疱性类天疱疮和寻常性天疱疮的发病趋势,特别是考虑到单个月内记录的住院人数。对一年中12个月的住院总人数进行了评估。此外,还对一些患者进行了血液化学测定和酶联免疫吸附测定,以评估抗体。关于疾病的严重程度,使用大疱性类天疱疮面积指数和天疱疮疾病面积指数评分系统。结果显示,季节和自身免疫状况等环境因素之间存在复杂的相互作用。
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引用次数: 0
Cutaneous squamous cell carcinoma of the lip successfully treated with rhenium-188 brachytherapy. 铼-188近距离放疗成功治疗唇部皮肤鳞状细胞癌。
IF 1.1 Q2 DERMATOLOGY Pub Date : 2023-06-14 eCollection Date: 2023-09-12 DOI: 10.4081/dr.2023.9560
Chiara Sabbadini, Federico Patta, Leda Lorenzon, Mohsen Farsad, Carla Nobile

Cutaneous squamous cell carcinoma (SCC) is the second most common form of skin cancer. In most cases, non-invasive SCC has a good prognosis and is curable by surgical resection. Nevertheless, a small percentage of patients pose specific management problems due to the technical difficulty of maintaining function and aesthetics because of the size or location of the tumor. An emerging therapeutic approach with high-dose brachytherapy using a nonsealed rhenium-188 resin, commercially known as Rhenium-Skin Cancer Therapy®, has been shown to be highly effective in non-invasive carcinoma, up to a thickness of 2-3 mm.

皮肤鳞状细胞癌(SCC)是癌症中第二常见的皮肤癌。在大多数情况下,无创SCC预后良好,可通过手术切除治愈。然而,由于肿瘤的大小或位置,维持功能和美观的技术困难,一小部分患者提出了特定的管理问题。一种新出现的高剂量近距离放射治疗方法,使用未密封的铼-188树脂,商业上称为铼皮肤癌症治疗®,已被证明对厚度达2-3毫米的非侵入性癌症非常有效。
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引用次数: 0
Cicatricial alopecia associated with pemphigus. 伴天疱疮的瘢痕性脱发。
IF 1.1 Q2 DERMATOLOGY Pub Date : 2023-06-14 eCollection Date: 2023-09-12 DOI: 10.4081/dr.2023.9653
Roberto D'Astolto, Chiara Zangrando, Claudio Feliciani

Pemphigus is a chronic autoimmune bullous disease associated with the production of autoantibodies directed against desmosomal proteins, such as desmogleins 1 and 3. Here, we present the case of an 83-year-old woman who was referred to us with suspicious cicatricial alopecia of the scalp and a small, eroded lesion on the forehead, previously labeled as atrophic actinic keratosis after a skin biopsy. In our clinic, after a careful examination of the case, we decided to perform two new skin biopsies of the scalp on suspicion of an inflammatory disease.

天疱疮是一种慢性自身免疫性大疱性疾病,与产生针对桥粒蛋白(如桥粒蛋白1和3)的自身抗体有关。在这里,我们介绍了一位83岁的女性,她被转介给我们,患有可疑的头皮瘢痕性脱发和前额的一个小的侵蚀性病变,之前在皮肤活检后被标记为萎缩性光化性角化病。在我们的诊所,在仔细检查了这个病例后,我们决定对怀疑患有炎症性疾病的头皮进行两次新的皮肤活检。
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引用次数: 0
Real-world use of dimethyl fumarate in patients with plaque psoriasis: a Delphi-based expert consensus. 富马酸二甲酯在斑块型银屑病患者中的实际应用:基于德尔菲的专家共识。
IF 1.1 Q2 DERMATOLOGY Pub Date : 2023-06-07 DOI: 10.4081/dr.2023.9613
Martina Burlando, Elena Campione, Aldo Cuccia, Giovanna Malara, Luigi Naldi, Francesca Prignano, Leonardo Zichichi

Dimethyl fumarate (DMF) was recently approved by the European Medicines Agency for systemic treatment of moderateto- severe chronic plaque psoriasis. Appropriate management of DMF treatment is required to achieve optimal clinical benefits. 7 dermatology experts gathered online for 3 meetings to identify consensus on the use of DMF in patient selection, drug dosage/titration, side effects management, and follow-up, with the aim to provide guidance on the use of DMF for psoriasis in clinical dermatological practice based on literature data and expert opinion. 20 statements were discussed and voted on using a facilitator- mediated modified Delphi methodology. Strong consensus was reached for all statements (agreement level of 100%). DMF treatment is characterized by dosage flexibility, sustained efficacy, high rates of drug survival, and low potential for drug-drug interactions. It can be used in a broad range of patients, including the elderly or those with comorbidities. Side effects (mainly gastrointestinal disorders, flushing, and lymphopenia) are frequently reported but are generally mild and transient and can be minimized by dosage adjustments and a slow titration schedule. Hematologic monitoring throughout the treatment course is required to reduce the risk of lymphopenia. This consensus document provides clinical dermatologists with answers on the optimal use of DMF to treat psoriasis.

富马酸二甲酯(DMF)最近被欧洲药品管理局批准用于中重度慢性斑块性银屑病的全身治疗。需要对DMF治疗进行适当的管理,以获得最佳的临床效益。7位皮肤科专家在线召开了3次会议,就DMF在患者选择、药物剂量/滴定、副作用管理和随访方面的使用达成共识,目的是根据文献数据和专家意见,为临床皮肤科实践中使用DMF治疗牛皮癣提供指导。对20个陈述进行了讨论,并对使用促进者介导的改进德尔菲方法进行了投票。所有陈述达成了强烈的共识(一致水平为100%)。DMF治疗的特点是剂量灵活,持续有效,药物存活率高,药物相互作用的可能性低。它可以用于广泛的患者,包括老年人或那些有合并症。副作用(主要是胃肠道疾病、潮红和淋巴细胞减少)经常被报道,但通常是轻微和短暂的,可以通过剂量调整和缓慢的滴定计划来最小化。在整个治疗过程中需要进行血液学监测,以减少淋巴细胞减少的风险。这份共识文件为临床皮肤科医生提供了DMF治疗牛皮癣的最佳使用答案。
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引用次数: 1
The first reported case of erythrodermic sarcoidosis with systemic involvement during COVID-19 vaccination. 首例在COVID-19疫苗接种期间全身性感染的红皮病结节病报告病例。
IF 1.1 Q2 DERMATOLOGY Pub Date : 2023-06-07 DOI: 10.4081/dr.2023.9636
Georgi Tchernev, Simona Kordeva, Heily Kirilova, Valentina Broshtilova

Post-vaccinal and parainfectious activation of the immunity with subsequent development of a certain immunological/skinimmunological disease is not rare in clinical practice. This concept is mentioned in relation to molecular/antigenic mimicry. To this day, the pathogenesis of sarcoidosis and sarcoid-type reactions remains a mystery. Moreover, they can be a warning sign of changes in tissue homeostasis, whether they are infectious, noninfectious- immunological, tumor-related, etc. We present a rare form of erythrodermic sarcoidosis with massive systemic involvement (pericarditis, supraventricular tachycardia, hepatitis, iritis/iridocyclitis, pulmonary fibrosis/bihilar lymphadenopathy, and arthritis) developed after receiving the ChadOx1-S vaccine for COVID- 19. Systemic immunosuppressive therapy with Methylprednisolone was introduced according to a scheme (in a reduction mode with an initial dose of 40 mg/day intravenously) in combination with topical Pimecrolimus 1% cream twice a day. Rapid improvement of the symptoms was observed within the first two days of treatment. According to the scientific literature, the presented patient turns out to be the first case of erythrodermic sarcoidosis (with systemic involvement), described as a side effect after vaccination and/or administration of a certain medicinal form.

在临床实践中,伴随某种免疫/皮肤免疫疾病发展的疫苗接种后和副感染免疫激活并不罕见。这个概念是在分子/抗原模仿中提到的。直到今天,结节病和结节型反应的发病机制仍然是一个谜。此外,它们可能是组织稳态变化的警告信号,无论它们是传染性的,非传染性的-免疫的,肿瘤相关的等等。我们报告了一种罕见的红皮病结节病,在接受针对COVID- 19的ChadOx1-S疫苗后,出现了大面积全身累及(心包炎、室上性心动过速、肝炎、虹膜炎/虹膜睫状体炎、肺纤维化/肝门淋巴结病和关节炎)。采用甲强的松龙进行全身免疫抑制治疗(初始剂量为40mg /天静脉注射),结合吡美莫司1%乳膏,每日2次。在治疗的头两天内观察到症状的迅速改善。根据科学文献,该患者是第一例红皮病结节病(全身受累),描述为接种疫苗和/或服用某种药物后的副作用。
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引用次数: 1
Human monkeypox coinfection with syphilis in an immunocompromised patient. 免疫功能低下患者猴痘合并梅毒。
IF 1.1 Q2 DERMATOLOGY Pub Date : 2023-06-07 DOI: 10.4081/dr.2022.9591
Irvin Ordoñez-González, Berenice López-Zamora, Gabriela Medina, Geraldine Vanessa Reyes-Navarro, Alberto Ordinola Navarro, María Pilar Cruz-Domínguez, Olga Vera-Lastra, Miguel Ángel Saavedra

Monkeypox is a viral zoonosis from the Poxviridae family that spreads at an unprecedented rate. It is transmitted through contact with skin lesions, respiratory droplets, body fluids, and sexual contact. The diverse presentation of the disease leads to misdiagnosis. Thus, clinicians should have a high index of suspicion, mainly with diseases with skin lesions. The most vulnerable group to developing this disease are individuals with risky sexual relationships, sexually transmitted infections, or human immunodeficiency virus/acquired immunodeficiency syndrome (HIV/AIDS). To date, only one case of coinfection with the monkeypox virus, syphilis, and HIV has been reported; however, no cases have been revealed in the Mexican territory. Herein we describe an unusual case of syphilis-monkeypox coinfection in an immunocompromised patient; despite his coinfection, he had a favorable prognosis. Furthermore, we attach allusive pictures of the natural evolution of dermatological lesions.

猴痘是一种来自痘病毒科的病毒性人畜共患病,以前所未有的速度传播。它通过接触皮肤损伤、呼吸道飞沫、体液和性接触传播。该病的多种表现导致误诊。因此,临床医生应该有高度的怀疑指数,主要是有皮肤病变的疾病。最易患此病的群体是有危险性性关系、性传播感染或人体免疫机能丧失病毒/后天免疫机能丧失综合症(艾滋病毒/艾滋病)的个人。迄今为止,仅报告了一例猴痘病毒、梅毒和艾滋病毒合并感染病例;然而,在墨西哥境内没有发现任何病例。在这里,我们描述了一个不寻常的情况下,梅毒-猴痘合并感染在免疫功能低下的病人;尽管他同时感染,但预后良好。此外,我们附上皮肤病变自然演变的暗示图片。
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引用次数: 3
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