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Experiences of patients with cutaneous manifestations of monoclonal gammopathy of undetermined significance (MGUS): Insights from the first disease-specific support group 意义未定的单克隆丙种球蛋白病(MGUS)皮肤表现患者的经历:来自首个特定疾病支持小组的启示
Pub Date : 2024-04-30 DOI: 10.1002/jvc2.427
Emily R. Gordon, Caroline Chen, Oluwaseyi Adeuyan, Brigit A. Lapolla, Megan H. Trager, Celine M. Schreidah, Lauren M. Fahmy, Larisa J. Geskin
<p>Monoclonal gammopathy of undetermined significance (MGUS) is a clonal plasma cell disorder characterized by monoclonal immunoglobulins or an abnormal ratio of free immunoglobulin light chains in blood or urine. Affecting 5% of adults aged 50 and older, MGUS is often asymptomatic and is typically incidentally diagnosed. In most cases, it requires only conservative observation and follow-up. However, MGUS may result in severe complications, such as significant cutaneous morbidity, which is not currently considered part of the diagnostic ‘requirement’ for multiple myeloma.<span><sup>1, 2</sup></span></p><p>Dermatologic manifestations linked to MGUS, which we named ‘gammopathic dermopathy’, include cutaneous light chain amyloidosis, POEMS syndrome, and scleromyxedema, among others.<span><sup>3-6</sup></span> Despite their existence, cutaneous MGUS manifestations remain poorly understood, leading to diagnostic delays and patient morbidity.<span><sup>7, 8</sup></span></p><p>While there are numerous multiple myeloma support groups, no official groups exist for those with MGUS. These patients struggle to find appropriate care and have difficulty obtaining effective medications because of the lack of diagnostic recognition, challenges with insurance coverage, and increased cost of medications. Thus, we organized the inaugural support group through Columbia Dermatology for patients with gammopathic dermopathy, titled ‘MGUS 4 Us’, to better understand the experiences of these patients.</p><p>Ten patients attended the meeting, sharing stories that highlighted common themes such as uncertainty and confusion about diagnosis, challenges in communicating diagnoses with families, difficulties in finding doctors with expertise, and struggles in obtaining effective treatment.</p><p>All 10 patients completed an optional survey with skin conditions reported including eczema, pyoderma gangrenosum, and scleromyxedema (Table 1). Notably, at least five patients experienced skin symptoms without an established diagnosis. Symptoms included itching (70%), pain (20%), rash (20%), and tightness (10%). Timelines varied, with one patient recently diagnosed with MGUS, five diagnosed 1–5 years ago, three diagnosed 5–10 years ago, and one diagnosed over 10 years ago. Two patients developed skin symptoms this year, three developed symptoms 1–5 years ago, two developed symptoms 5–10 years ago, two developed symptoms over 10 years ago, and one was unknown.</p><p>Of the 10 patients, six received treatment for skin symptoms, including topical steroids (4), multiple myeloma drugs (lenalidomide/ixazomib, 2), hydroxychloroquine (1), intravenous immunoglobulin (1), oral steroids (1), antibiotics (1), and dupilumab (1). While one patient reported feeling ‘a lot better’ after treatment, three felt ‘a little better’, two reported no change, and four did not receive treatment. The survey also revealed that most patients had dermatologists, oncologists, haematologists or rheumatologists invo
LJG 曾担任 Helsinn Group、J&amp;J、Mallinckrodt、Kyowa Kirin、Soligenix、Innate、Incyte、Trillium、Merck、BMS 和 Stratpharma 的研究员和/或获得这些公司的研究支持;是 Mallinckrodt 和 Recordati 的发言人;是 SciTech 和 Celsyntec 的科学顾问委员会成员。EG、CC、OA、BL、MT、LF、CS 没有需要声明的利益冲突。
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引用次数: 0
Full-body skin examination in screening for cutaneous malignancy: A focus on concealed sites and the practices of international dermatologists 皮肤恶性肿瘤筛查中的全身皮肤检查:关注隐蔽部位和国际皮肤科医生的做法
Pub Date : 2024-04-29 DOI: 10.1002/jvc2.437
James P. Pham, Nicholas Allen, Phoebe Star, Anne Cust, Pascale Guitera, Ashfaq A. Marghoob, John Paoli, Iris Zalaudek, Annika Smith

Background

Full-body skin examination (FSE) is fundamental to the diagnosis of cutaneous malignancy but may not always include concealed site examination (CSE).

Objectives

To determine the approach of international dermatologists to CSE during FSE and examine influencing factors, barriers and attitudes toward CSE.

Methods

Members of the International Dermoscopy Society were surveyed using an online 12-question survey disseminated via email.

Results

There were 706 completed responses among 1249 unique clicks to the survey, representing a completion rate of 56.5%. Fifty-four percent of respondents reported always examining the breasts, while 52.8%, 18.8%, and 11.8% always examined the scalp, oral, and anogenital mucosa, respectively. The most frequent reason for examining concealed sites was patient concern, whilst common reasons for not examining concealed sites included low incidence of pathology and concern regarding allegations of sexual misconduct.

Conclusions

Our findings allude to the need for international consensus guidelines regarding the conduct and inclusion of concealed or sensitive sites in routine FSE. This is essential to define clinician responsibilities, inform patient expectations of care, and thereby mitigate potential medicolegal repercussions.

背景 全身皮肤检查(FSE)是诊断皮肤恶性肿瘤的基础,但可能并不总是包括隐蔽部位检查(CSE)。 目的 确定国际皮肤科医生在进行全身皮肤检查(FSE)时对隐蔽部位检查(CSE)的态度,并研究影响因素、障碍和对 CSE 的态度。 方法 通过电子邮件对国际皮肤镜学会成员进行在线调查,调查包含 12 个问题。 结果 在1249次点击调查中,有706次完成了回复,完成率为56.5%。54%的受访者表示经常检查乳房,而经常检查头皮、口腔和肛门粘膜的受访者分别占52.8%、18.8%和11.8%。检查隐蔽部位最常见的原因是病人担心,而不检查隐蔽部位的常见原因包括病理发生率低和担心性行为不端指控。 结论 我们的研究结果表明,有必要就隐蔽或敏感部位的行为和纳入常规 FSE 制定国际共识指南。这对于明确临床医生的责任、告知患者对护理的期望,从而减轻潜在的医学法律影响至关重要。
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引用次数: 0
Impact of atopic dermatitis lesion locations and extent on patient burden: A real-world study 特应性皮炎皮损位置和程度对患者负担的影响:真实世界研究
Pub Date : 2024-04-26 DOI: 10.1002/jvc2.413
Eric Simpson, Peter Lio, Evangeline Pierce, Angel Cronin, Robert R. McLean, Thomas Eckmann, Amber Reck Atwater, Zach Dawson, Jonathan I. Silverberg
<div> <section> <h3> Background</h3> <p>Atopic dermatitis (AD) is associated with patient burden, but few studies describe the anatomic distribution of the disease or the impact of number of lesion locations.</p> </section> <section> <h3> Objectives</h3> <p>To describe lesion locations and assess the relationship between the number of lesion locations (disease extent) and disease burden in patients with AD.</p> </section> <section> <h3> Methods</h3> <p>This cross-sectional study included adults with dermatologist- or dermatology practitioner-diagnosed AD enroled in the CorEvitas AD Registry (2020–2021) who initiated systemic therapy within 12 months prior to or at enrolment or had moderate-to-severe AD (vIGA-AD® ≥3 and EASI ≥12) at enrolment. Thirteen areas of lesion involvement were assessed using a body map, and numbers of lesion locations were categorised as: 0, 1, 2–3, 4–6 and ≥7. Demographics, disease characteristics, PROs by number of lesion locations were descriptively compared using effect sizes (ES). The ES thresholds for small, medium, and large differences, respectively, were 0.10, 0.30, and 0.50 for phi (categorical outcomes) and 0.10, 0.25 and 0.40 for Cohen's f (continuous outcomes).</p> </section> <section> <h3> Results</h3> <p>Among 1211 patients, lesion involvement was most frequent on the arms (69.5%) and lower limbs (61.7%). A total of 10.6%, 9.3%, 20.1%, 26.3% and 33.8% of patients had 0, 1, 2–3, 4–6 and ≥7 lesion locations, respectively. Current use of systemic (≥81.2%) and topical ( ≥74.7%) therapies was common, irrespective of lesion location. Disease severity increased with number of lesion locations: mean total BSA (ES = 1.17), EASI (ES = 1.11), and SCORAD (ES = 1.21). vIGA-AD ≥3 was observed in 28.3%, 45.3%, 78.0%, and 93.9% of patients with 1, 2–3, 4–6 and ≥7 locations, respectively (ES = 0.63). Greater number of lesion locations was associated with worse PROs: mean POEM (ES = 0.57), sleep loss (ES = 0.41), peak pruritus (ES = 0.50), DLQI (ES = 0.40), and ADCT (ES = 0.53). Uncontrolled AD (ADCT ≥7) was observed in 48.2%, 52.9%, 70.4%, 81.6% of patients with 1, 2–3, 4–6 and ≥7 locations, respectively (ES = 0.42).</p> </section> <section> <h3> Conclusions</h3> <p>AD lesions were reported for each body area assessed. Greater number of lesion locations was associated with increased disease severity, poor disease control, and decreased quality of life. Patients experienced substantial disease burden regardless of number of les
背景 特应性皮炎(AD)与患者的负担有关,但很少有研究描述这种疾病的解剖分布或皮损位置数量的影响。 目的 描述特应性皮炎患者的皮损位置并评估皮损位置数量(疾病范围)与疾病负担之间的关系。 方法 该横断面研究纳入了CorEvitas AD注册(2020-2021年)中由皮肤科医生或皮肤科执业医师确诊的成人AD患者,这些患者在注册前或注册时的12个月内开始接受系统治疗,或在注册时患有中重度AD(vIGA-AD® ≥3和EASI ≥12)。使用体表图评估了13个病变受累区域,病变位置的数量分为:0、1、2-3、4、5、6、7、8、9、10、11、12:0、1、2-3、4-6 和≥7。使用效应大小(ES)对人口统计学、疾病特征、PROs 和病变部位数量进行描述性比较。phi(分类结果)的小、中、大差异 ES 临界值分别为 0.10、0.30 和 0.50,Cohen's f(连续结果)的小、中、大差异 ES 临界值分别为 0.10、0.25 和 0.40。 结果 在 1211 名患者中,病变最常累及手臂(69.5%)和下肢(61.7%)。分别有10.6%、9.3%、20.1%、26.3%和33.8%的患者病变部位为0、1、2-3、4-6和≥7个。无论皮损位置如何,目前使用全身疗法(≥81.2%)和局部疗法(≥74.7%)的情况都很普遍。vIGA-AD≥3的患者比例分别为28.3%、45.3%、78.0%和93.9%,病变位置分别为1、2-3、4-6和≥7个(ES = 0.63)。病变位置越多,患者的PRO越差:平均POEM(ES = 0.57)、睡眠质量下降(ES = 0.41)、峰值瘙痒(ES = 0.50)、DLQI(ES = 0.40)和ADCT(ES = 0.53)。48.2%、52.9%、70.4%、81.6%的患者观察到未控制的 AD(ADCT ≥7),位置分别为 1、2-3、4-6 和 ≥7(ES = 0.42)。 结论 所评估的每个身体部位都有 AD 病变。病变部位越多,疾病严重程度越高,疾病控制能力越差,生活质量越低。无论病变部位的数量多少,患者都承受着巨大的疾病负担。
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引用次数: 0
Recalcitrant pityriasis rubra pilaris in a Middle Eastern patient and arguments for early anti-IL-23 targeting 一名中东患者顽固的红斑狼疮和早期抗IL-23靶向治疗的论据
Pub Date : 2024-04-26 DOI: 10.1002/jvc2.435
Mohammed N. Al-Abdulla, Wadha Al-Shafi, Hanof Ahmed, Anh Jochebeth, Febu Joy, Shahd Younis, Mahir Petkar, Joerg Buddenkotte, Martin Steinhoff

Pityriasis rubra pilaris (PRP) is a rare, chronic cutaneous inflammatory disorder of keratinization affecting adults, children and patients with HIV. The pathogenesis of PRP is not fully understood with clinical presentations, and severity remains highly variable. Current treatment modalities for PRP result in recalcitrant disease with potentially unfavourable therapeutic side effects and low tolerability. Due to the rarity of this condition, limited data on treatment efficacies and established management guidelines are lacking. The psychological burden of PRP is detrimental to the quality of life of patients affected with PRP and remains a persistent gap of knowledge. Here, we provide a review of the literature, summarizing new developments in the treatment of PRP and a case report of a patient treated successfully with the anti-interleukin (IL)-23p19 antibody Risankizumab with sustained clinical improvement. Risankizumab appears to be an effective and safe treatment for PRP in Asian-Arabic patients. Further studies are required to assess the efficacy, safety and tolerability of newer targeted therapies for severe PRP.

红斑性瘙痒症(PRP)是一种罕见的慢性角化性皮肤炎症,成人、儿童和艾滋病患者均可患病。PRP 的发病机制尚不完全清楚,临床表现和严重程度也千差万别。目前针对 PRP 的治疗方法会导致疾病反复发作,并可能带来不利的治疗副作用和较低的耐受性。由于这种疾病的罕见性,有关治疗效果和既定管理指南的数据十分有限。PRP 带来的心理负担不利于 PRP 患者的生活质量,这仍然是一个长期的知识空白。在此,我们对文献进行了综述,总结了 PRP 治疗的新进展,并报告了一例使用抗白细胞介素(IL)-23p19 抗体 Risankizumab 成功治疗并持续改善临床症状的病例。利桑珠单抗似乎是治疗亚洲-阿拉伯患者 PRP 的一种有效而安全的方法。还需要进一步的研究来评估新的靶向疗法对严重 PRP 的疗效、安全性和耐受性。
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引用次数: 0
Attitudes and practices of inflammatory bowel disease patients towards skin cancer risk and sun protection 炎症性肠病患者对皮肤癌风险和防晒的态度和做法
Pub Date : 2024-04-26 DOI: 10.1002/jvc2.425
Maeve Herlihy, Eve Blake, Pauline Carroll, Garry Courtney

Background

Skin cancer is the most common form of cancer in Ireland with over 13,000 new cases diagnosed annually. People with inflammatory bowel disease are at higher risk of developing skin cancer.

Objectives

The aim of this study is to assess inflammatory bowel disease (IBD) patients' awareness around skin cancer risk and photoprotection practices and to identify gaps in patient education regarding skin cancer prevention in IBD patients.

Methods

IBD patients >18 years old were invited to fill out a questionnaire while waiting for their outpatient appointment.

Results

A total of 87 patients (52% women) with IBD (Crohn's disease 62.8%, UC 35%,) were included. A total of 38 patients were diagnosed before age 25 (44%). Most patients were on active treatment for their IBD (96.5%, n = 85). Four patients had a personal history, and seven patients had a family history of skin cancer. Nearly half of participants (47.6%) were unaware or unsure of an increased risk of skin cancer associated with IBD and 42.5% were unaware or unsure of an increased risk with some IBD treatments. Behaviours around sun-cream were generally good, however, other sun protective methods were suboptimal.

Conclusions

Only half (52.4%) of IBD patients surveyed were aware of a link between skin cancer and IBD. Skin cancer knowledge was generally good, however, photoprotection practices were suboptimal. This underscores the role of healthcare professionals in re-enforcing sun safety information and skin cancer education in the IBD population.

背景 皮肤癌是爱尔兰最常见的癌症,每年新确诊病例超过 13,000 例。炎症性肠病患者患皮肤癌的风险较高。 本研究旨在评估炎症性肠病(IBD)患者对皮肤癌风险和光防护措施的认识,并找出 IBD 患者在皮肤癌预防方面的患者教育差距。 方法 邀请年满 18 岁的 IBD 患者在门诊候诊时填写问卷。 结果 共纳入 87 名 IBD 患者(52% 为女性)(克罗恩病 62.8%,UC 35%)。共有 38 名患者在 25 岁之前确诊(占 44%)。大多数患者正在接受 IBD 的积极治疗(96.5%,n = 85)。四名患者有个人皮肤癌病史,七名患者有家族皮肤癌病史。近一半的参与者(47.6%)不知道或不确定 IBD 会增加患皮肤癌的风险,42.5% 的参与者不知道或不确定某些 IBD 治疗会增加患皮肤癌的风险。使用防晒霜的行为普遍良好,但其他防晒方法则不尽人意。 结论 在接受调查的 IBD 患者中,只有一半(52.4%)知道皮肤癌与 IBD 之间存在联系。患者对皮肤癌的认知度普遍较高,但防晒措施却不够理想。这凸显了医护人员在加强 IBD 患者防晒安全信息和皮肤癌教育方面的作用。
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引用次数: 0
Reflectance confocal microscopy and clinical evaluation of a product containing Silybum marianum fruit extract in monotherapy for acne vulgaris treatment: A prospective study 采用反射共聚焦显微镜和临床评估含水飞蓟果实提取物的产品对寻常型痤疮的单一疗法治疗效果:前瞻性研究
Pub Date : 2024-04-25 DOI: 10.1002/jvc2.419
Elisete I. Crocco, Renata O. Alves, Gustavo S. M. Carvalho, Rebeca R. A. Silva, Ricardo S. B. Silva, Karina B. C. V. Calbucci, Ana L. F. Coutinho, Christiano S. Andrade, Juliana C. T. Braga

Background

Acne is a common pilosebaceous unit chronic inflammatory disease, with a multifactorial pathogenesis. It involves several processes: increased sebum production, alteration of the follicular keratinization process and bacterial colonization by Cutibacterium acnes.

Objectives

To evaluate the effectiveness of reflectance confocal microscopy (RCM) in evaluating the reduction of mild to severe acne lesions in patients treated with an investigational product containing Silybum marianum fruit extract (SMFE).

Methods

Subjects previously examined for counting acne lesions were topically treated in the face, with an investigational product containing SMFE in monotherapy over the course of 60 days (n = 31). They were examined for counting acne lesions. Efficacy and quality of life, tolerability, and safety assessments were performed. The infundibular diameter (ID) measurement of normal and acne skin areas was analyzed using RCM.

Results

The RCM data statistical analysis allowed us to identify two ID patterns, 300–750 µm, with a reduction of 33.33% (p < 0.05) and 900–1050 µm with 73.16% (p < 0.05) reduction after 60 days. The noninflammatory lesions had a mean variation of −44.51% and −67.82% after 30 and 60 days, respectively (t test: p < 0.05 and p < 0.001 after Bonferroni's correction). The inflammatory lesions had a mean variation of −69.31% and −79.40% after 30 and 60 days (t test: p < 0.05 and p = 0.118 after Bonferroni's correction).

Conclusions

The authors demonstrated the effectiveness of the monotherapy scheme with an investigational product containing SMFE through the statistically significant results obtained by the RCM in acneic and nonacneic skin, in addition to the reduction in the number of noninflammatory at the end of the 60 days of clinical follow-up. Furthermore, the monotherapy scheme with the investigational product proved safe, with no serious adverse events during the entire clinical study. In summary, the use of RCM proved to be effective in assessing the therapeutic response in patients with moderate to severe acne.

背景痤疮是一种常见的皮脂腺单位慢性炎症性疾病,其发病机制是多因素的。它涉及几个过程:皮脂分泌增加、毛囊角质化过程改变和痤疮棒状杆菌的细菌定植。 目的 评估反射共聚焦显微镜(RCM)在评估使用含水飞蓟果实提取物(SMFE)的研究产品治疗患者的轻度至重度痤疮皮损减少情况方面的有效性。 方法 对之前进行过痤疮皮损计数检查的受试者进行面部局部治疗,在 60 天的治疗过程中使用含有水飞蓟果提取物的研究产品进行单一疗法(n = 31)。他们接受了痤疮皮损计数检查。对疗效、生活质量、耐受性和安全性进行了评估。使用 RCM 分析了正常皮肤区域和痤疮皮肤区域的皮下内径 (ID) 测量值。 结果 通过 RCM 数据统计分析,我们确定了两种 ID 模式:300-750 微米,60 天后减少 33.33% (p < 0.05);900-1050 微米,减少 73.16% (p < 0.05)。非炎性病变在 30 天和 60 天后的平均变化率分别为-44.51%和-67.82%(t 检验:经 Bonferroni 校正后,p < 0.05 和 p < 0.001)。炎症病变在 30 天和 60 天后的平均变化率分别为-69.31%和-79.40%(t 检验:p < 0.05,经 Bonferroni 校正后 p = 0.118)。 结论 作者通过 RCM 在痤疮和非痤疮皮肤上获得的显著统计结果,以及在 60 天临床随访结束时非炎症数量的减少,证明了使用含 SMFE 的研究产品的单一疗法方案的有效性。此外,在整个临床研究期间,该研究产品的单一疗法方案证明是安全的,没有出现严重的不良反应。总之,事实证明,使用 RCM 可以有效评估中重度痤疮患者的治疗反应。
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引用次数: 0
Clinical features of drug reaction with eosinophilia and systemic symptoms: A retrospective study in a reference centre of dermatology in Madagascar 伴有嗜酸性粒细胞增多和全身症状的药物反应的临床特征:马达加斯加一家皮肤病参考中心的回顾性研究
Pub Date : 2024-04-25 DOI: 10.1002/jvc2.438
Fandresena Arilala Sendrasoa, Bienvenu Chrismael Imbelona, Tsiory Iarintsoa Razafimaharo, Lala Ramarozatovo, Fahafahantsoa Rapelanoro Rabenja

Background

Drug reaction with eosinophilia and systemic symptoms (DRESS) is drug-induced hypersensitivity reaction that can have fatal complications. Three sets of diagnostic criteria have been proposed, however, consensus is lacking.

Objectives

This study aimed to describe the causative agents, severity, and the clinical course of patients with DRESS in Antananarivo, Madagascar.

Methods

A cross-sectional study was conducted in patients seen for DRESS, in the department of dermatology at the University Hospital Antananarivo, Madagascar from 2014 to 2022. European Registry of Severe Cutaneous Adverse Reactions (RegiSCAR) criteria was used for the diagnosis. Demographic data, latency periods, clinical and laboratory findings, culprit drugs, and outcomes were assessed.

Results

A total of 24 patients were included. Fifteen patients were female. The three most common culprit drugs were carbamazepine (17 patients), phenobarbital (2 patients), and ampicilline (2 patients). Median onset time (interquartile range) was 20 days (range: 7–62 days). Skin rash was present in all patients, and fever in 18 patients. A total of 12 patients showed two or more internal organs involved. Liver and kidney injuries were the most common visceral manifestation. All patients had eosinophilia. Fifteen patients received systemic corticosteroids. Two mortality cases were reported due to DRESS-related fulminant liver failure and to nosocomial respiratory infection. Mortality was associated with higher eosinophilia (p = 0.03), higher creatininemia (p = 0.04), and shorter time of latency (p = 0.04).

Conclusions

Our study show that DRESS is associated with severe morbidity. Early detection and withdrawal of the culprit dru gis crucial to save life and reduce morbidity.

背景 伴有嗜酸性粒细胞增多和全身症状的药物反应(DRESS)是由药物诱发的超敏反应,可引起致命的并发症。目前已提出三套诊断标准,但尚未达成共识。 目的 本研究旨在描述马达加斯加塔那那利佛 DRESS 患者的致病因子、严重程度和临床病程。 方法 对马达加斯加塔那那利佛大学医院皮肤科 2014 年至 2022 年因 DRESS 就诊的患者进行横断面研究。诊断采用欧洲严重皮肤不良反应登记处(RegiSCAR)标准。对人口统计学数据、潜伏期、临床和实验室检查结果、罪魁祸首药物以及治疗效果进行了评估。 结果 共纳入 24 名患者。其中 15 名患者为女性。三种最常见的罪魁祸首药物分别是卡马西平(17 例)、苯巴比妥(2 例)和氨苄西林(2 例)。中位发病时间(四分位数间距)为 20 天(范围:7-62 天)。所有患者均出现皮疹,18 名患者发烧。共有 12 名患者的两个或两个以上内脏器官受累。肝脏和肾脏损伤是最常见的内脏表现。所有患者都有嗜酸性粒细胞增多。15 名患者接受了全身皮质类固醇治疗。有两例死亡病例的报告,原因分别是与DRESS相关的暴发性肝衰竭和院内呼吸道感染。死亡率与嗜酸性粒细胞增多(p = 0.03)、肌酐血症升高(p = 0.04)和潜伏期缩短(p = 0.04)有关。 结论 我们的研究表明,DRESS 与严重的发病率有关。早期发现并停用罪魁祸首药物对于挽救生命和降低发病率至关重要。
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引用次数: 0
Questions a patient with moderate to severe atopic dermatitis would like to be asked: Data from a qualitative study conducted by anthropologists 中重度特应性皮炎患者希望被问到的问题:来自人类学家定性研究的数据
Pub Date : 2024-04-24 DOI: 10.1002/jvc2.423
Judith Nicogossian, Stéphane Héas, Claire Thénié, Margaux Noel, Laurent Misery, Sebastien Barbarot, Fabienne Martin Juchat

Background

A considerable number of patients with moderate to severe atopic dermatitis (AD) are undertreated and have inadequate control of their symptoms leading to a hight patient dissatisfaction during their care. The VECUDA study composed of VECU and DA for french word lived and atopic dermatitis was conducted by anthropologists in cooperation with dermatologists, aimed to better understand the complexity of the care pathway and the physician-patient relationship by questioning patients with moderate to severe AD.

Objectives

The aim was to identify patient's needs and to propose concrete ways of improving the doctor-patient relationship by focusing on the narrative of their affects. Co-designed easy-to-use tools were proposed by these patients.

Methods

The exploratory qualitative study involved 24 patients with moderate-to-severe AD. The study was conducted in two stages: a codesign phase with four patients, and a deepening phase with all patients (24), supported by a multidisciplinary scientific committee. During group interviews, conducted using creativity tools, patients' stories related to their experiences of the disease and its management were collected.

Results

By studying the patients' affect stories, researchers were able to identify the needs of patients who might be 1. emotional (better consideration of their experiences); 2. informational (more information about their disease and treatments); 3. participatory (being involved in their care: therapeutic alliance). Mechanisms of therapeutic dissatisfaction and satisfaction were mapped into vicious and virtuous circles. Patients also offered concrete and easy-to-use tools. We are presenting here the grid of questions these patients would like to be asked during their doctor's appointment: the Doctor-Facing Questions Grid that these patients would like to be asked during a consultation.

Conclusions

By facilitating the identification of patients' needs/expectations, these tools would help guide the clinician's discussion and management in a few questions. The VECUDA study confirms the value of considering patients' perspective as a lever for improving the doctor-patient relationship.

相当多的中重度特应性皮炎(AD)患者治疗不足,症状控制不力,导致患者对治疗过程极不满意。由人类学家与皮肤科医生合作开展的 VECUDA 研究由 VECU 和 DA 组成,旨在通过询问中重度异位性皮炎患者,更好地了解护理途径和医患关系的复杂性。这项探索性定性研究涉及 24 名中重度注意力缺失症患者。这项探索性定性研究涉及 24 名中重度注意力缺失症患者。研究分两个阶段进行:在多学科科学委员会的支持下,与四名患者共同设计阶段和与所有患者(24 名)共同深化阶段。通过研究患者的情感故事,研究人员确定了患者的需求:1.情感需求(更好地考虑他们的经历);2.信息需求(更多有关疾病和治疗的信息);3.参与需求(参与护理:治疗联盟)。治疗不满意和满意的机制被划分为恶性循环和良性循环。患者还提供了具体易用的工具。我们在此介绍这些患者希望在就诊时被问到的问题网格:这些患者希望在就诊时被问到的面向医生的问题网格。通过促进确定患者的需求/期望,这些工具将有助于指导临床医生通过几个问题进行讨论和管理。VECUDA 研究证实了将病人的观点作为改善医患关系的杠杆的价值。
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引用次数: 0
Upadacitinib for management of alopecia areata and rheumatoid arthritis: Letter to the editor 乌达帕替尼用于治疗斑秃和类风湿性关节炎:致编辑的信
Pub Date : 2024-04-24 DOI: 10.1002/jvc2.433
Ieva Lingytė, Paula Kaminskienė, Arūnas Petkevičius, Asta Baranauskaitė
<p>We present the case of a 54-year-old woman with a 14-year history of seropositive rheumatoid arthritis (RA) and 12 years of patchy alopecia areata (AA). RA presented with typical symmetric arthritis of small joints of the hands, morning stiffness lasting for more than 1 h, high rheumatoid factor (49.5 kU/L), and anti-cyclic citrullinated peptide antibodies (153.14 kU/L). The treatment of RA involved oral methotrexate at a dose of 17.5 mg per week, along with nonsteroidal anti-inflammatory drugs, prescribed in 2009. Hair loss began 2 years later, and the patient consulted a dermatologist who diagnosed AA. Since methotrexate was suitable for both diseases, no additional systemic AA treatment was prescribed at that time, and topical treatment for the scalp (0.05% clobetasol propionate and 5% minoxidil) was added. However, the topical treatment showed no effect and was discontinued after a few years. Methotrexate was changed to leflunomide at a daily dose of 20 mg due to its ineffectiveness for arthritis at the end of 2011. As RA remained active with a high Disease Activity Score-28 (DAS-28) index, reaching up to 5.43, leflunomide was stopped, and an inhibitor of tumour necrosis factor-alpha etanercept at 50 mg per week, was prescribed in 2013 and continued for 10 years. During this treatment, low disease activity of RA was maintained with short-term exacerbations, and no significant side effects were observed. The DAS-28 index ranged from 1.32 to 3.47. However, AA persisted throughout this period without significant improvement or deterioration. An exacerbation of RA and AA was noticed at the beginning of 2023, leading to the decision to stop etanercept and to start therapy with the selective Janus kinase (JAK) 1 inhibitor upadacitinib at a dose of 15 mg daily. Upadacitinib was prescribed as it was the only JAK inhibitor reimbursed in Lithuania at that time. During the visit, trichoscopy of the scalp was performed and showed broken hairs, black and yellow dots, vellus hairs, with a Severity of Alopecia Tool (SALT) score of 51 (Figure 1a). A significant improvement in RA was observed within 3 months, with the DAS-28 decreasing from 2.21 to 1.18. The hair regrew completely after 6 months with the SALT score of 0 and with no evidence of disease activity on trichoscopy (Figure 1b,c). No side effects were observed with upadacitinib, and laboratory tests performed every 3 months returned normal results.</p><p>AA is associated with autoimmune diseases such as RA due to the similar involvement of the JAK and signal transducer and activator of transcription signalling pathway (JAK/STAT).<span><sup>1, 2</sup></span> Cytokines such interferon-γ, interleukin-2 (IL-2), interleukin-7 (IL-7) and interleukin-15 (IL-15) are believed to play an important role in the pathogenesis of AA. These cytokines activate certain JAK molecules, leading to the phosphorylation of the corresponding domains of the STAT pathway. This phosphorylation ultimately leads to the transcr
我们为您介绍一例 54 岁女性的病例,她的类风湿性关节炎(RA)血清阳性病史长达 14 年,斑片状脱发(AA)病史长达 12 年。RA 表现为典型的手部小关节对称性关节炎,晨僵持续 1 小时以上,类风湿因子高(49.5 kU/L),抗环瓜氨酸肽抗体(153.14 kU/L)。治疗类风湿关节炎的方法是口服甲氨蝶呤,剂量为每周 17.5 毫克,同时服用非甾体类抗炎药(2009 年处方)。两年后开始脱发,患者咨询了皮肤科医生,医生诊断其为 AA。由于甲氨蝶呤适用于这两种疾病,因此当时没有再对 AA 进行系统治疗,而是增加了头皮局部治疗(0.05% 氯倍他索丙酸酯和 5%米诺地尔)。然而,外用治疗没有效果,几年后停用。由于甲氨蝶呤对关节炎的疗效不佳,2011 年底,患者将甲氨蝶呤改为来氟米特,每日剂量为 20 毫克。由于RA仍处于活动期,疾病活动度评分-28(DAS-28)指数高达5.43,因此停用来氟米特,并于2013年开始使用肿瘤坏死因子-α抑制剂依那西普(etanercept),每周50毫克,持续治疗10年。在治疗期间,RA 的疾病活动度维持在较低水平,仅有短期加重,未观察到明显的副作用。DAS-28 指数从 1.32 到 3.47 不等。然而,AA 在整个治疗期间持续存在,没有明显改善或恶化。2023年初,患者发现RA和AA病情加重,因此决定停用依那西普,开始使用选择性Janus激酶(JAK)1抑制剂乌达替尼治疗,剂量为每天15毫克。之所以开具乌达替尼处方,是因为它是当时立陶宛唯一可报销的JAK抑制剂。就诊期间,对患者的头皮进行了毛囊镜检查,结果显示患者有断发、黑点和黄点、绒毛,脱发严重程度工具(SALT)评分为51分(图1a)。3 个月后,RA 明显好转,DAS-28 从 2.21 降至 1.18。6 个月后,头发完全长出,SALT 评分为 0,三镜检查未发现疾病活动迹象(图 1b、c)。1、2 干扰素-γ、白细胞介素-2(IL-2)、白细胞介素-7(IL-7)和白细胞介素-15(IL-15)等细胞因子被认为在 AA 的发病机制中发挥了重要作用。这些细胞因子会激活某些 JAK 分子,导致 STAT 通路的相应结构域发生磷酸化。3 JAK 抑制剂有望成为治疗 AA 的新方法。2022 年 6 月,美国食品和药物管理局(FDA)批准巴利替尼(baricitinib)用于治疗严重 AA,2023 年 6 月,FDA 又批准了另一种 JAK 抑制剂--利妥昔尼(ritlecitinib)。乌达帕替尼是一种选择性 JAK-1 抑制剂,可阻断多种炎症细胞因子(包括 IL-2、IL-6、IL-7 和 IL-15)的信号传导,抑制这两种疾病常见的炎症、6 文献中已发表了数例每日服用15或20毫克达帕替尼治疗AA合并特应性皮炎或克罗恩病患者的成功病例。7, 8 在这封信中,我们提供了证据,证明每日服用15毫克达帕替尼可有效治疗RA患者的AA:Ieva Lingytė:工作构思;数据分析和解释,文章起草。Paula Kaminskienė:数据分析和解释、文章起草。Arūnas Petkevičius:对文章进行严格修改,最终批准发布的版本。Asta Baranauskaitė:作者声明无利益冲突。本手稿中的所有患者均已书面知情同意参与本研究,并同意将其去标识化、匿名化的汇总数据及其病例详情(包括照片)用于发表。伦理批准:不适用。
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引用次数: 0
The complexity of the care pathway for a patient with moderate to severe atopic dermatitis seeking a new treatment: Data from a qualitative anthropological study 中重度特应性皮炎患者寻求新疗法的护理路径的复杂性:人类学定性研究数据
Pub Date : 2024-04-24 DOI: 10.1002/jvc2.424
Judith Nicogossian, Stéphane Héas, Claire Thénié, Margaux Noel, Laurent Misery, Sébastien Barbarot, Fabienne Martin Juchat
<div> <section> <h3> Background</h3> <p>A considerable number of patients with moderate to severe atopic dermatitis (AD) are undertreated and have inadequate control of their symptoms leading to a hight patient dissatisfaction during their care. The VECUDA study, was conducted by anthropologists in cooperation with dermatologists, aimed to better understand the complexity of the care pathway and the physician-patient relationship by questioning patients with moderate to severe AD.</p> </section> <section> <h3> Objectives</h3> <p>The aim was to identify patient's needs and to propose concrete ways of improving the doctor-patient relationship by focusing on the narrative of their affects. Co-designed easy-to-use tools were proposed by these patients.</p> </section> <section> <h3> Methods</h3> <p>The exploratory qualitative study involved 24 patients with moderate-to-severe AD. The study was conducted in two stages: a co-design phase with 4 patients, and a deepening phase with all patients (24), supported by a multidisciplinary scientific committee. During group interviews, conducted using creativity tools, patients' stories related to their experiences of the disease and its management were collected.</p> </section> <section> <h3> Results</h3> <p>The VECUDA study, conducted by anthropologists in collaboration with dermatologists and focusing on the experiences of these patients and their journey, described mechanisms of dissatisfaction related to therapeutic management from the point of view of patients with moderate to severe atopic dermatitis. Factors that could generate dissatisfaction included the complexity of the care pathway. During this exploratory study emerged an unexpected patient figure, who had a “feeling of a being chosen” in access to a new treatment, the first biologic in the treatment of AD. We are interested here in the experience of the patient's care pathway.</p> </section> <section> <h3> Conclusions</h3> <p>The VECUDA study confirms the value of considering patients' perspective as a lever for improving the doctor-patient relationship. The overall conclusion is based on the observation of a gap between patient expectations and treatment decisions made by some HCPs, which may lead to unsatisfactory scenarios. Analysing these data opens up a promising research avenue, showing that patients not only confer a therapeutic function to their care pathway, but also a symbolic value. The description of the complexity of the care pathway c
背景相当多的中重度特应性皮炎(AD)患者治疗不当,症状控制不力,导致患者对治疗过程极不满意。人类学家与皮肤科医生合作开展了 VECUDA 研究,旨在通过询问中重度特应性皮炎患者,更好地了解护理途径的复杂性和医患关系。 目的 通过关注患者对自身影响的叙述,确定患者的需求,并提出改善医患关系的具体方法。这些患者提出了共同设计的易于使用的工具。 方法 这项探索性定性研究涉及 24 名中重度注意力缺失症患者。研究分两个阶段进行:与 4 名患者共同设计阶段,以及在多学科科学委员会的支持下与所有患者(24 名)共同深化阶段。在使用创造性工具进行的小组访谈中,收集了患者有关疾病及其管理经验的故事。 结果 VECUDA 研究由人类学家与皮肤科医生合作开展,重点关注这些患者的经历及其心路历程,从中重度特应性皮炎患者的角度描述了与治疗管理有关的不满机制。可能产生不满的因素包括护理路径的复杂性。在这项探索性研究中,出现了一位意想不到的患者,他在接受一种新疗法(治疗过敏性皮炎的第一种生物制剂)时有一种 "被选择的感觉"。在此,我们对患者的治疗经历感兴趣。 结论 VECUDA 研究证实了将患者的观点作为改善医患关系杠杆的价值。总体结论的依据是观察到患者的期望与某些保健医生的治疗决定之间存在差距,这可能会导致令人不满意的情况。对这些数据的分析开辟了一条前景广阔的研究途径,表明患者不仅赋予其护理路径以治疗功能,还赋予其象征价值。对护理路径复杂性的描述可以比作真正的 "起步之旅"。患者在寻求新的治疗方法时对其护理路径所赋予的象征性价值分析是一种创新方法,值得通过定性和定量研究进一步探讨。
{"title":"The complexity of the care pathway for a patient with moderate to severe atopic dermatitis seeking a new treatment: Data from a qualitative anthropological study","authors":"Judith Nicogossian,&nbsp;Stéphane Héas,&nbsp;Claire Thénié,&nbsp;Margaux Noel,&nbsp;Laurent Misery,&nbsp;Sébastien Barbarot,&nbsp;Fabienne Martin Juchat","doi":"10.1002/jvc2.424","DOIUrl":"10.1002/jvc2.424","url":null,"abstract":"&lt;div&gt;\u0000 \u0000 \u0000 &lt;section&gt;\u0000 \u0000 &lt;h3&gt; Background&lt;/h3&gt;\u0000 \u0000 &lt;p&gt;A considerable number of patients with moderate to severe atopic dermatitis (AD) are undertreated and have inadequate control of their symptoms leading to a hight patient dissatisfaction during their care. The VECUDA study, was conducted by anthropologists in cooperation with dermatologists, aimed to better understand the complexity of the care pathway and the physician-patient relationship by questioning patients with moderate to severe AD.&lt;/p&gt;\u0000 &lt;/section&gt;\u0000 \u0000 &lt;section&gt;\u0000 \u0000 &lt;h3&gt; Objectives&lt;/h3&gt;\u0000 \u0000 &lt;p&gt;The aim was to identify patient's needs and to propose concrete ways of improving the doctor-patient relationship by focusing on the narrative of their affects. Co-designed easy-to-use tools were proposed by these patients.&lt;/p&gt;\u0000 &lt;/section&gt;\u0000 \u0000 &lt;section&gt;\u0000 \u0000 &lt;h3&gt; Methods&lt;/h3&gt;\u0000 \u0000 &lt;p&gt;The exploratory qualitative study involved 24 patients with moderate-to-severe AD. The study was conducted in two stages: a co-design phase with 4 patients, and a deepening phase with all patients (24), supported by a multidisciplinary scientific committee. During group interviews, conducted using creativity tools, patients' stories related to their experiences of the disease and its management were collected.&lt;/p&gt;\u0000 &lt;/section&gt;\u0000 \u0000 &lt;section&gt;\u0000 \u0000 &lt;h3&gt; Results&lt;/h3&gt;\u0000 \u0000 &lt;p&gt;The VECUDA study, conducted by anthropologists in collaboration with dermatologists and focusing on the experiences of these patients and their journey, described mechanisms of dissatisfaction related to therapeutic management from the point of view of patients with moderate to severe atopic dermatitis. Factors that could generate dissatisfaction included the complexity of the care pathway. During this exploratory study emerged an unexpected patient figure, who had a “feeling of a being chosen” in access to a new treatment, the first biologic in the treatment of AD. We are interested here in the experience of the patient's care pathway.&lt;/p&gt;\u0000 &lt;/section&gt;\u0000 \u0000 &lt;section&gt;\u0000 \u0000 &lt;h3&gt; Conclusions&lt;/h3&gt;\u0000 \u0000 &lt;p&gt;The VECUDA study confirms the value of considering patients' perspective as a lever for improving the doctor-patient relationship. The overall conclusion is based on the observation of a gap between patient expectations and treatment decisions made by some HCPs, which may lead to unsatisfactory scenarios. Analysing these data opens up a promising research avenue, showing that patients not only confer a therapeutic function to their care pathway, but also a symbolic value. The description of the complexity of the care pathway c","PeriodicalId":94325,"journal":{"name":"JEADV clinical practice","volume":"3 4","pages":"1325-1333"},"PeriodicalIF":0.0,"publicationDate":"2024-04-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1002/jvc2.424","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140659158","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
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JEADV clinical practice
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