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Netherton syndrome in a Bulgarian patient : Presentation of a case and an update of therapeutic options. 内瑟顿综合征在保加利亚患者:一个病例的介绍和治疗方案的更新。
IF 0.9 Q2 MEDICINE, GENERAL & INTERNAL Pub Date : 2023-09-01 DOI: 10.1007/s10354-022-00999-y
Simona Atanasova Kordeva, Ilia Batashki, Georgi Tchernev

Comel-Netherton syndrome, or Netherton syndrome (NS), is a rare chronic genetic skin condition affecting the daily life of patients, which often results in poorly developed social skills and anxiety. Genetic predisposition plays a key role alongside the clinical findings, and clinicians must be aware of it as it can mimic other well-known skin conditions. Diagnosis is challenging both clinically and histologically. Clinically, it can mimic a severe form of atopic dermatitis, psoriasiform dermatitis overlapping with atopic dermatitis, or erythrokeratodermia variabilis. The difficulties in making histological diagnosis are similar, and it is often necessary to take several biopsies in order to clarify the diagnosis. Although retinoids are used for both psoriasis, erythrokeratodermia variabilis, and other congenital forms of keratodermia, the recommended treatment doses are different. This often results in poor treatment outcome. We present a 16-year-old patient previously diagnosed as erythrokeratodermia variabilis and treated with little to no improvement. Systemic therapy with acitretin 10 mg daily, local pimecrolimus 1%, emollients, and bilastine 20 mg once daily was initiated. Due to the limited application of retinoids and the difficulties in achieving permanent remission, modern medicine is faced with the challenge of seeking innovative therapeutic solutions. New hopes are placed on targeted or anti-cytokine therapy, based on inhibiting the inflammatory component of the disease. This article is mainly focused on innovative therapeutic options, including modern medications such as dupilumab, infliximab, secukinumab, anakinra, omalizumab, and others.

Comel-Netherton综合征或内瑟顿综合征(NS)是一种罕见的慢性遗传性皮肤病,影响患者的日常生活,通常导致社交技能发育不良和焦虑。遗传易感性在临床发现中起着关键作用,临床医生必须意识到这一点,因为它可以模仿其他众所周知的皮肤状况。诊断是具有挑战性的临床和组织学。临床上,它可以模拟严重形式的特应性皮炎,银屑病样皮炎与特应性皮炎重叠,或变异性红角化皮炎。在做组织学诊断的困难是相似的,往往需要采取多次活检,以明确诊断。虽然类维生素a可用于牛皮癣、变异性红角化皮病和其他先天性角化皮病,但推荐的治疗剂量是不同的。这往往导致治疗效果不佳。我们提出一个16岁的病人以前诊断为变异性红细胞角化皮病和治疗很少或没有改善。开始全身治疗:阿维a素10 mg /天,局部吡美莫司1%,润肤剂,bilastine 20 mg /天一次。由于类维生素a的应用有限,难以实现永久缓解,现代医学面临着寻求创新治疗方案的挑战。新的希望寄托在基于抑制疾病炎症成分的靶向或抗细胞因子治疗上。本文主要关注创新的治疗选择,包括现代药物,如dupilumab、英夫利昔单抗、secukinumab、anakinra、omalizumab等。
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引用次数: 0
[Microcannular liposuction in tumescent anesthesia in lipedema patients: an analysis of 519 liposuctions]. [微环形吸脂术在肿胀麻醉下治疗脂肪水肿患者:519例吸脂分析]。
IF 0.9 Q2 MEDICINE, GENERAL & INTERNAL Pub Date : 2023-09-01 Epub Date: 2023-06-14 DOI: 10.1007/s10354-023-01017-5
Alicia Kodim, Uwe Wollina

This is a retrospective analysis of all lipedema patients treated by tumescent liposuction at our department in the years 2007-2021: We performed 519 liposuctions in 178 patients with a mean age of 45 ± 15.5 years. By the stage of lipedema the mean age increased significantly, what underlines the concept of lipedema as a chronic progressive disorder. Three-thirds of patients reported at least one comorbidity. The most common were arterial hypertension (32.58%), obesity (24.16%), and hypothyroidism (20.79%). We removed a mean lipoaspirate volume of 4905 ± 2800 mL. A major target for treatment is pain reduction. All patients reported at least a 50% pain reduction after liposuction, while 96 achieved a pain reduction ≥ 90%. The pre-operative pain intensity (p = 0.000) and the lipedema stage (p = 0.032) exerted a significant impact on absolute pain reduction. There was no association of pain reduction to volume loss. The post-operative rate of adverse events was 2.89%. Liposuction in tumescent anesthesia is an effective and safe method to reduce both pain and volume in patients with lipedema.

这是对2007-2021年我科所有接受肿胀抽脂治疗的脂水肿患者的回顾性分析:我们对178名平均年龄为45岁的患者进行了519次抽脂 ± 15.5岁。到了脂肪水肿阶段,平均年龄显著增加,这突出了脂肪水肿作为一种慢性进行性疾病的概念。三分之三的患者报告至少有一种共病。最常见的是动脉高压(32.58%)、肥胖(24.16%)和甲状腺功能减退(20.79%) ± 2800 mL。治疗的主要目标是减轻疼痛。所有患者均报告抽脂后疼痛减轻至少50%,而96名患者的疼痛减轻≥50% 90%。术前疼痛强度(p = 0.000)和脂水肿期(p = 0.032)对绝对疼痛减轻产生了显著影响。疼痛减轻与体积减少没有关联。术后不良事件发生率为2.89%。肿胀麻醉下抽脂是一种有效、安全的方法,可以减轻脂水肿患者的疼痛和体积。
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引用次数: 0
Malignant melanoma in a patient with hidrotic ectodermal dysplasia. 一名发育不良的外胚层发育不良患者的恶性黑色素瘤。
IF 0.9 Q2 MEDICINE, GENERAL & INTERNAL Pub Date : 2023-09-01 Epub Date: 2022-06-20 DOI: 10.1007/s10354-022-00940-3
Hristo Petrov Dobrev, Tsvetana Ivanova Abadjieva, Teodor Ivanov Aleksiev, Zlatina Georgieva Ivanova, Nina Ivanova Vutova
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引用次数: 0
Response to R. Mungmumpuntipanip and V. Wiwantikit : Correspondence to "Post-SARS-CoV-2 vaccination COVID toes and fingers". 对R. Mungmumpuntipanip和V. wiwanantikit的回应:与“后sars - cov -2疫苗COVID脚趾和手指”的对应。
IF 0.9 Q2 MEDICINE, GENERAL & INTERNAL Pub Date : 2023-09-01 DOI: 10.1007/s10354-022-00973-8
Uwe Wollina
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引用次数: 0
Alopecia associated with dimethyl fumarate treatment for multiple sclerosis. 富马酸二甲酯治疗多发性硬化症与脱发相关。
IF 0.9 Q2 MEDICINE, GENERAL & INTERNAL Pub Date : 2023-09-01 DOI: 10.1007/s10354-023-01007-7
Ana Abičić, Ivan Adamec, Mario Habek

After 2 weeks of treatment, a woman with multiple sclerosis treated with dimethyl fumarate developed alopecia. Considering the adverse events, the therapy was discontinued, leading to alopecia regression during the next 3 months. Although the precise mechanism has not been completely elucidated, glutathione depletion or downregulation of aerobic glycolysis are considered to be potential reasons for hair loss induction. The incidence and mechanism of this uncommon adverse reaction to dimethyl fumarate should be further investigated.

治疗2周后,一名多发性硬化症患者接受富马酸二甲酯治疗后出现脱发。考虑到不良事件,停止治疗,导致在接下来的3个月内脱发消退。虽然确切的机制尚未完全阐明,谷胱甘肽耗竭或有氧糖酵解下调被认为是诱导脱发的潜在原因。这种罕见的富马酸二甲酯不良反应的发生率和机制有待进一步研究。
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引用次数: 1
Hemoptoe, thin-walled lung cysts, and spontaneous pneumothorax are features of metastatic cutaneous angiosarcoma : A case report. 血趾、薄壁肺囊肿和自发性气胸是转移性皮肤血管肉瘤的特征。
IF 0.9 Q2 MEDICINE, GENERAL & INTERNAL Pub Date : 2023-09-01 DOI: 10.1007/s10354-022-00934-1
Iurii Mykoliuk, Martin Zacharias, Oliver Sankin, Jörg Lindenmann, Freyja-Maria Smolle-Juettner

We present a case of bilateral cystic lung metastases originating from cutaneous angiosarcoma (cAS) of the scalp in a 73-year-old man. He presented with hemoptysis and recurrent bilateral pneumothorax. The clinical, radiological, and histological features and a potential pathophysiological mechanism of pulmonary changes in cutaneous angiosarcoma are discussed.

我们报告一个73岁男性头皮皮肤血管肉瘤(cAS)的双侧囊性肺转移病例。他表现为咯血和复发性双侧气胸。本文讨论了皮肤血管肉瘤肺部病变的临床、放射学和组织学特征以及潜在的病理生理机制。
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引用次数: 1
Onychomadesis in a COVID-19 patient. COVID-19患者的甲发育。
IF 0.9 Q2 MEDICINE, GENERAL & INTERNAL Pub Date : 2023-09-01 DOI: 10.1007/s10354-022-00988-1
Zlatina Georgieva Ivanova, Teodor Ivanov Aleksiev, Hristo Petrov Dobrev

We report the case of a 67-year-old woman who developed onychomadesis on 9 of her fingers 2 months after recovering from COVID-19, with subsequent full nail regrowth after 4 months. The development of onychomadesis in COVID-19 is probably related to inhibition of nail proliferation due to fever, direct viral damage, or an inflammatory process associated with endothelial damage and obliterative microangiopathy in the nail matrix area. Clinicians should be aware of nail changes and actively seek them out in patients with COVID-19.

我们报告了一名67岁女性的病例,她在COVID-19康复2个月后出现了9个手指的甲发育,随后在4个月后全指甲再生。COVID-19患者的甲发育可能与发热、直接病毒损伤或与甲基质区内皮损伤和闭塞性微血管病变相关的炎症过程抑制指甲增殖有关。临床医生应该意识到COVID-19患者的指甲变化,并积极寻找它们。
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引用次数: 0
Post-SARS-CoV-2 vaccination COVID toes and fingers correspondence. sars - cov -2疫苗接种后,COVID脚趾和手指对应。
IF 0.9 Q2 MEDICINE, GENERAL & INTERNAL Pub Date : 2023-09-01 DOI: 10.1007/s10354-022-00971-w
Rujittika Mungmunpuntipantip, Viroj Wiwanitkit
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引用次数: 1
Anaphylactic reaction to carboplatin diagnosed by skin testing-a reliable tool in platinum-based immediate-type hypersensitivity reactions. 通过皮肤试验诊断卡铂过敏反应——一种基于铂的即时型超敏反应的可靠工具。
IF 0.9 Q2 MEDICINE, GENERAL & INTERNAL Pub Date : 2023-09-01 DOI: 10.1007/s10354-022-00938-x
Elias Marquart, Ahmad Jalili, Nadine Mothes-Luksch, Stephan N Wagner, Tamar Kinaciyan

Immediate-type hypersensitivity reactions (IHRs) to carboplatin (CA) are most commonly reported in ovarian cancer patients. A 54-year-old woman with stage IV melanoma suffering from metastasis in the entire right lower extremity was presented to our allergy outpatient clinic for diagnostic work-up due to an anaphylactic reaction with palmoplantar erythema, conjunctivitis along with facial erythema, and an incipient decrease in blood pressure during a chemotherapy regimen with dacarbazine and carboplatin upon re-administration. A subsequently carried out allergological work-up with skin testing (ST) revealed CA to be the culprit drug, whereas cisplatin (CI) was confirmed to be a safe alternative for the patient for following treatments. Here, we report a case of an IHR to carboplatin in a melanoma patient, with CI serving as a safe alternative diagnosed by skin testing.

对卡铂(CA)的立即型超敏反应(IHRs)是卵巢癌患者中最常见的报道。一名54岁女性,患IV期黑色素瘤,整个右下肢发生转移,因掌跖红斑、结膜炎伴面部红斑的过敏反应,以及再次给予达卡巴嗪和卡铂化疗后血压初期下降,来到我们的过敏门诊进行诊断检查。随后进行的过敏检查和皮肤试验(ST)显示CA是罪魁祸首药物,而顺铂(CI)被证实是患者后续治疗的安全选择。在这里,我们报告了一例黑色素瘤患者卡铂的IHR, CI作为一种安全的替代方法,通过皮肤试验进行诊断。
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引用次数: 1
Post-SARS-CoV-2 vaccination COVID toes and fingers : Two case reports and a short literature review. 接种 SARS-CoV-2 疫苗后的 COVID 脚趾和手指:两份病例报告和简短的文献综述。
IF 1.1 Q2 MEDICINE, GENERAL & INTERNAL Pub Date : 2023-09-01 Epub Date: 2022-06-23 DOI: 10.1007/s10354-022-00944-z
Uwe Wollina
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引用次数: 0
期刊
Wiener medizinische Wochenschrift
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