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National clinical reaudit on managing adults with bullous pemphigoid 2024 highlighting shifting clinical practices. 国家临床审核管理成人大疱性类天疱疮2024强调转变临床实践。
Q3 Medicine Pub Date : 2025-11-06 eCollection Date: 2025-12-01 DOI: 10.1093/skinhd/vzaf063
Ravi Ramessur, Hayley Smith, Zoe C Venables, Tanya Tumbeva, M Firouz Mohd Mustapa, David A R de Berker

Background: Bullous pemphigoid (BP) is a chronic, autoimmune, blistering disorder that predominantly affects older adults and is associated with significant morbidity and treatment challenges. The British Association of Dermatologists (BAD) clinical guideline for managing people with BP was published in 2012; a national clinical audit was undertaken in 2018. This 2024 reaudit evaluates changes in clinical practice, including diagnostic methods, treatment strategies and documentation standards.

Objectives: To reassess compliance with BAD audit standards, compare findings with the 2018 audit and identify trends in BP management.

Methods: Over 9 weeks in 2024, BAD members submitted data for 450 cases of BP from 77 centres across the UK. Audit standards included documentation of comorbidities, osteoporosis risk management, patient satisfaction and systemic treatment monitoring.

Results: The reaudit identified a shift in diagnostic practices, an increased proportion of severe baseline disease and continued gaps in -osteoporosis risk documentation. The use of doxycycline as a primary treatment has increased significantly since 2018.

Conclusions: The findings highlight both progress and persistent challenges in BP management. Improved documentation and greater adherence to osteoporosis management guidelines remain priorities for future practice.

背景:大疱性类天疱疮(BP)是一种慢性自身免疫性水疱疾病,主要影响老年人,具有显著的发病率和治疗挑战。2012年,英国皮肤科医师协会(BAD)发布了管理BP患者的临床指南;2018年开展全国临床审计。2024年的重新审核评估了临床实践的变化,包括诊断方法、治疗策略和文件标准。目的:重新评估对BAD审计标准的符合性,将调查结果与2018年的审计结果进行比较,并确定BP管理的趋势。方法:在2024年的9周内,BAD成员提交了来自英国77个中心的450例BP病例的数据。审核标准包括合并症、骨质疏松风险管理、患者满意度和系统治疗监测的记录。结果:重新审核确定了诊断实践的转变,严重基线疾病的比例增加,骨质疏松症风险文件的持续空白。自2018年以来,强力霉素作为主要治疗药物的使用显著增加。结论:研究结果强调了BP管理的进展和持续的挑战。改进文献和更严格地遵守骨质疏松管理指南仍然是未来实践的重点。
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引用次数: 0
Intranasal corticosteroids: a promising new approach for adults with chronic idiopathic urticaria linked to aeroallergens - a double-blind randomized clinical trial. 鼻内皮质类固醇:治疗与空气过敏原相关的成人慢性特发性荨麻疹的有希望的新方法-一项双盲随机临床试验
Q3 Medicine Pub Date : 2025-11-05 eCollection Date: 2025-12-01 DOI: 10.1093/skinhd/vzaf078
Hassan Mohtadi, Hossein Esmaeilzadeh, Aida Askarisarvestani, Seyed Hesamodin Nabavizadeh, Soheila Alyasin

Background: Chronic idiopathic urticaria (CIU) is a debilitating condition characterized by recurrent hives, itching and angioedema lasting for more than 6 weeks without an identifiable cause. It significantly impacts quality of life, causing physical discomfort and emotional distress. CIU's complex pathophysiology involves immune interactions, mast cells and histamine release, with aeroallergens potentially exacerbating symptoms in sensitized patients. Current treatments primarily involve antihistamines, but many patients experience inadequate relief, prompting the exploration of alternative therapies. Intranasal corticosteroids, typically used for allergic rhinitis, may offer potential benefits for CIU, although evidence of their efficacy is limited.

Objectives: To investigate the effect of intranasal corticosteroids in adults with CIU who had a positive skin prick test for aeroallergens.

Methods: In this double-blind placebo-controlled trial, 69 adults with CIU sensitized to aeroallergens received budesonide 32 µg per actuation, 2 sprays per nostril twice daily or identical placebo for 6 months while maintained on a stable single-agent nonsedating H1 antihistamine. Participants were randomly assigned to receive either budesonide or placebo. Outcomes included changes in urticaria symptoms and Urticaria Severity Score and quality of life. The study was registered with the Iranian Registry of Clinical Trials (IRCT 20231229060563N1).

Results: At baseline, both groups were comparable. Post-treatment, participants in the corticosteroid group showed a greater reduction in Urticaria Activity Score (mean difference = 4.25, P = 0.02). No significant difference was found in chronic urticaria quality of life questionnaire scores (mean difference = 4.74, P = 0.35).

Conclusions: Intranasal corticosteroids were effective in reducing urticaria symptoms but did not offer significant benefits over placebo with regard to overall quality of life. The results highlight the complexity of CIU management and suggest a potential placebo effect.

背景:慢性特发性荨麻疹(CIU)是一种衰弱性疾病,其特征是反复发作的荨麻疹、瘙痒和血管性水肿持续6周以上,原因不明。它会严重影响生活质量,导致身体不适和情绪困扰。CIU复杂的病理生理涉及免疫相互作用、肥大细胞和组胺释放,空气过敏原可能加剧致敏患者的症状。目前的治疗主要涉及抗组胺药,但许多患者感觉缓解不足,促使探索替代疗法。鼻内皮质类固醇,通常用于过敏性鼻炎,可能为CIU提供潜在的益处,尽管其有效性的证据有限。目的:探讨鼻内皮质类固醇对空气过敏原皮肤点刺试验阳性的成年CIU患者的影响。方法:在这项双盲安慰剂对照试验中,69名对空气过敏原敏感的成年CIU患者接受布地奈德每次32 μ g,每次鼻孔2次,每天2次或相同的安慰剂,持续6个月,同时维持稳定的单药非镇静H1抗组胺药。参与者被随机分配接受布地奈德或安慰剂。结果包括荨麻疹症状、荨麻疹严重程度评分和生活质量的变化。该研究已在伊朗临床试验登记处注册(IRCT 20231229060563N1)。结果:在基线时,两组具有可比性。治疗后,皮质类固醇组的参与者在荨麻疹活动评分方面表现出更大的降低(平均差异= 4.25,P = 0.02)。两组慢性荨麻疹患者生活质量问卷得分差异无统计学意义(平均差异4.74,P = 0.35)。结论:鼻内皮质类固醇在减轻荨麻疹症状方面是有效的,但在总体生活质量方面并没有比安慰剂提供显著的益处。结果强调了CIU管理的复杂性,并提示了潜在的安慰剂效应。
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引用次数: 0
Unravelling the causal link between gut microbiota and acne risk using a genetic approach. 利用遗传方法揭示肠道微生物群与痤疮风险之间的因果关系。
Q3 Medicine Pub Date : 2025-11-05 eCollection Date: 2025-12-01 DOI: 10.1093/skinhd/vzaf077
Fangyuan Cao, Natalia S Ogonowski, Santiago Díaz-Torres, Brittany L Mitchell, Puya Gharhakhani, Nicholas G Martin, Michael A Simpson, Jue-Sheng Ong, Miguel E Rentería

Background: Acne is a common inflammatory dermatological condition that can have detrimental psychological consequences due to its visible lesions and scarring. Recent studies suggest a potential role of gut microbiota in acne development; however, the evidence remains inconclusive and might be subject to various confounders.

Objectives: To investigate the causal relationship between gut microbiota and acne.

Methods: This study investigated the causal relationship between gut microbiota and acne using a two-sample Mendelian randomization (MR) approach with large-scale genome-wide association study summary statistics. To ascertain the direction of causality and the independent effect of gut microbiota, reverse MR and multivariable MR accounting for dietary phenotypes were performed.

Results: Higher abundances of the Actinobacteria phylum and class, Bifidobacteriales order, Bifidobacteriaceae family and Bifidobacterium -genus were associated with a reduced risk of acne [odds ratios (ORs) ranging from 0.54 to 0.63]. In contrast, higher levels of the Gastranaerophilales order, Streptococcaceae family and Streptococcus genus were positively associated with an increased risk of acne (OR 1.12-1.36). Notably, associations for Bifidobacterium and its upstream taxa remained robust even after accounting for dietary factors.

Conclusions: These findings provide further evidence of a causal relationship between gut microbial composition and acne, highlighting the role of gut microbiota in developing more targeted and possibly less harmful alternatives to current acne management strategies.

背景:痤疮是一种常见的炎症性皮肤病,由于其可见的病变和疤痕,可产生有害的心理后果。最近的研究表明,肠道微生物群在痤疮发展中的潜在作用;然而,证据仍然不确定,可能受到各种混杂因素的影响。目的:探讨肠道菌群与痤疮的因果关系。方法:本研究采用双样本孟德尔随机化(MR)方法和大规模全基因组关联研究汇总统计,研究肠道微生物群与痤疮之间的因果关系。为了确定因果关系的方向和肠道微生物群的独立影响,进行了考虑饮食表型的反向MR和多变量MR。结果:放线菌门和纲、双歧杆菌目、双歧杆菌科和双歧杆菌属的丰度越高,痤疮的风险越低[比值比(or)从0.54到0.63]。相反,较高水平的嗜气胃菌目、链球菌科和链球菌属与痤疮风险增加呈正相关(OR为1.12-1.36)。值得注意的是,即使考虑到饮食因素,双歧杆菌及其上游分类群的关联仍然很强。结论:这些发现为肠道微生物组成与痤疮之间的因果关系提供了进一步的证据,强调了肠道微生物群在开发更有针对性和可能危害更小的替代方案方面的作用,以替代当前的痤疮管理策略。
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引用次数: 0
Genetic syndromes in paediatric alopecia areata: a systematic review. 遗传综合征在儿科斑秃:系统回顾。
Q3 Medicine Pub Date : 2025-11-05 eCollection Date: 2025-12-01 DOI: 10.1093/skinhd/vzaf080
Megan Park, Emma Price, Cathryn Sibbald

Background: A wide variation of phenotypes is displayed by individuals with alopecia areata (AA), especially in the paediatric population.

Objectives: To systematically search published studies to identify paediatric syndromes with AA and their clinical features, and to summarize the current state of their genetic elucidation.

Methods: In accordance with the PRISMA guidelines, a systematic search of MEDLINE, Embase, CENTRAL and PubMed databases was performed. All original case reports, case series and observational studies describing AA in children (aged <18 years) with monogenic or chromosomal syndromes were included. Further searches in OMIM and Orphanet, and reviews, clinical guidelines and basic science studies were used to retrieve additional comprehensive information on each syndrome.

Results: After title and abstract screening of 1426 studies, and full-text review of 224 studies, 64 met the inclusion criteria and are summarized in this review. Overall, the search identified 33 genetic syndromes with paediatric AA. Prevalence estimates were available for 79% (n = 26/33) of syndromes, with 45% (n = 15/33) of syndromes presenting in fewer than 1/1 000 000 individuals. Sixty-seven per cent (n = 22/33) of syndromes were fully genetically elucidated; 12% (n = 4/33) were partially elucidated; 9% (n = 3/33) were not genetically elucidated; and 12% (n = 4/33) were syndromes with chromosomal abnormalities. Seventy-nine per cent (n = 26/33) of syndromes were described by only one report, while 21% (n = 7/33) were described in multiple independent reports.

Conclusions: Despite the limited knowledge of these syndromes, this review provides insights into the range of genetic syndromes with paediatric AA and their clinical features, facilitating early prediction, diagnosis and personalized treatments.

背景:斑秃(AA)个体表现出广泛的表型变化,特别是在儿科人群中。目的:系统检索已发表的研究,以确定儿童AA综合征及其临床特征,并总结其遗传学研究的现状。方法:按照PRISMA指南,系统检索MEDLINE、Embase、CENTRAL和PubMed数据库。所有描述儿童AA的原始病例报告、病例系列和观察性研究结果:经过对1426项研究的标题和摘要筛选,以及224项研究的全文综述,64项研究符合纳入标准,并在本综述中进行总结。总的来说,研究确定了33种与儿科AA相关的遗传综合征。可获得79% (n = 26/33)综合征的患病率估计,其中45% (n = 15/33)的综合征出现在少于1/ 100万人中。67% (n = 22/33)的综合征在基因上被完全阐明;12% (n = 4/33)部分阐释;9% (n = 3/33)未被基因鉴定;12% (n = 4/33)为染色体异常综合征。79% (n = 26/33)的综合征仅被一份报告描述,21% (n = 7/33)的综合征被多个独立报告描述。结论:尽管对这些综合征的认识有限,但本综述提供了对儿童AA遗传综合征范围及其临床特征的深入了解,有助于早期预测、诊断和个性化治疗。
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引用次数: 0
Bound by tradition: severe pediculosis capitis in a breastfeeding mother with religious headwear. 受传统束缚:一位戴着宗教头饰的哺乳母亲患有严重的头癣。
Q3 Medicine Pub Date : 2025-11-05 eCollection Date: 2025-12-01 DOI: 10.1093/skinhd/vzaf079
Varun H, Hanumanthaiah H C, Adarshlata Singh, Bhushan Madke, Lamiya Latheef, Prerit Sharma

A 25-year-old breastfeeding woman from rural India presented with a 2-month history of intense scalp pruritus, formication and fever. Examination revealed a severe Pediculus humanus capitis infestation involving her scalp, face and religious headscarf, with extensive live lice, nits, pustules, excoriations, crusted papules and dermatopathic lymphadenopathy. Treatment with permethrin 1% lotion and weekly oral ivermectin for 4 weeks, in conjunction with broad-spectrum antibiotics and supportive care, resulted in near-complete eradication. This case represents one of the most severe presentations of pediculosis capitis documented, demonstrating how cultural practices can impede early detection and complicate management.

一名来自印度农村的25岁母乳喂养妇女出现2个月的严重头皮瘙痒、过敏和发烧史。检查发现严重的人头蒂感染,包括头皮、面部和宗教头巾,并伴有大量活虱、虱子、脓疱、擦伤、结痂丘疹和皮肤病性淋巴结病。用1%氯菊酯洗剂和每周口服伊维菌素治疗4周,再加上广谱抗生素和支持性护理,几乎完全根除了该病。本病例是记录在案的最严重的头弓根病之一,表明文化习俗如何阻碍早期发现并使管理复杂化。
{"title":"Bound by tradition: severe pediculosis capitis in a breastfeeding mother with religious headwear.","authors":"Varun H, Hanumanthaiah H C, Adarshlata Singh, Bhushan Madke, Lamiya Latheef, Prerit Sharma","doi":"10.1093/skinhd/vzaf079","DOIUrl":"https://doi.org/10.1093/skinhd/vzaf079","url":null,"abstract":"<p><p>A 25-year-old breastfeeding woman from rural India presented with a 2-month history of intense scalp pruritus, formication and fever. Examination revealed a severe <i>Pediculus humanus capitis</i> infestation involving her scalp, face and religious headscarf, with extensive live lice, nits, pustules, excoriations, crusted papules and dermatopathic lymphadenopathy. Treatment with permethrin 1% lotion and weekly oral ivermectin for 4 weeks, in conjunction with broad-spectrum antibiotics and supportive care, resulted in near-complete eradication. This case represents one of the most severe presentations of pediculosis capitis documented, demonstrating how cultural practices can impede early detection and complicate management.</p>","PeriodicalId":74804,"journal":{"name":"Skin health and disease","volume":"5 6","pages":"482-485"},"PeriodicalIF":0.0,"publicationDate":"2025-11-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12648546/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145643799","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}
引用次数: 0
Autosomal recessive woolly hair syndrome: a series of eight patients in an Indian population. 常染色体隐性毛毛综合征:一系列的八个病人在印度人口。
Q3 Medicine Pub Date : 2025-11-05 eCollection Date: 2025-12-01 DOI: 10.1093/skinhd/vzaf082
Vijay Somani, Anirudh Somani, Ashwini Annabathula

Woolly hair is an uncommon disorder of the hair shaft, characterized by tightly coiled scalp hair often accompanied by varying grades of hypotrichosis. Woolly hair involving the entire scalp can be syndromic when associated with various anomalies involving the heart, nervous system, liver, gastrointestinal organs and so on, and is designated as nonsyndromic when it occurs in isolation without any systemic involvement. Nonsyndromic woolly hair can be autosomal dominant or autosomal recessive. We hereby present a series of eight cases of autosomal recessive woolly hair (ARWH) seen in an Indian population in the last 12 years. Their clinical presentation, hair characteristics and response to treatment is described.

毛毛是一种罕见的毛干疾病,其特征是头皮头发紧密卷曲,常伴有不同程度的毛少症。累及整个头皮的毛毛,当与涉及心脏、神经系统、肝脏、胃肠等器官的各种异常有关时,可以是综合征性的;当孤立发生而不累及全身时,被指定为非综合征性的。非综合征性羊毛可为常染色体显性或常染色体隐性。我们在此提出一系列的8例常染色体隐性羊毛(ARWH)在过去的12年印度人口看到。描述了他们的临床表现,头发特征和对治疗的反应。
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引用次数: 0
An unusual presentation in relapse case of histoid leprosy. 麻风病复发病例的不寻常表现。
Q3 Medicine Pub Date : 2025-10-28 eCollection Date: 2025-12-01 DOI: 10.1093/skinhd/vzaf067
Mahesh Mathur, Sumit Paudel, Nabita Bhattarai, Sandhya Regmi, Sambidha Karki, Sharad Shrestha

Histoid leprosy (HL) is a very rare and highly infectious variant of lepromatous leprosy, presenting as skin-coloured, succulent nodules and plaques on apparently healthy skin. It is histologically characterized by a dense bundle of histiocytes arranged in storiform manner. Usually, it occurs in patients with leprosy who relapse after dapsone monotherapy or inadequate antileprotic treatment; it can even arise de novo. We present a case of umbilicated presentation in a patient with a case of relapse HL, even after adequate treatment with multibacillary multidrug therapy for 12 months. Although rare, the higher load of lepra bacilli in these cases makes it a matter of concern, and challenging for leprologists to make early diagnosis and treatment.

组织样麻风病(HL)是一种非常罕见且传染性很强的麻风病变种,在表面健康的皮肤上表现为皮肤颜色的肉质结节和斑块。它的组织学特征是一束致密的组织细胞呈故事状排列。通常发生在接受氨苯砜单药治疗或抗麻风治疗不充分后复发的麻风患者;它甚至可以从头开始。我们提出一个病例脐状呈现在一个复发的HL患者的情况下,即使经过充分的治疗多菌多药治疗12个月。虽然罕见,但在这些病例中较高的麻风杆菌负荷使其成为一个令人关注的问题,并且对麻风学家进行早期诊断和治疗具有挑战性。
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引用次数: 0
A qualitative review of Fitzpatrick skin types featured on the front covers of the British Association of Dermatologists journals. 一篇关于菲茨帕特里克皮肤类型的定性综述,刊登在英国皮肤病学会期刊的封面上。
Q3 Medicine Pub Date : 2025-10-24 eCollection Date: 2025-12-01 DOI: 10.1093/skinhd/vzaf059
Brian Nolan

The British Association of Dermatology (BAD) has gone to notable efforts to ensure adequate representation of darker skin tones within its journal. The Association has frequently highlighted the lack of awareness of presentations of hair, skin and nail pathologies in minority groups, and the contents of its journals - British Journal of Dermatology, Clinical and Experimental Dermatology and Skin Health and Disease - are representative of the significant work it has done to enhance physician awareness of darker skin tone pathologies. We explored the role of a front page of a medical journal. Our qualitative analysis of journal covers suggests front cover representation to be an ample opportunity for the BAD to continue to promote darker skin tone pathology awareness.

英国皮肤病学协会(BAD)已经做出了显著的努力,以确保其期刊中有足够的深色肤色代表。该协会经常强调,少数群体对头发、皮肤和指甲的病理表现缺乏认识,其期刊《英国皮肤病学杂志》、《临床和实验皮肤病学杂志》和《皮肤健康与疾病杂志》的内容代表了该协会为提高医生对深色肤色病理的认识所做的重要工作。我们探讨了医学杂志头版的作用。我们对杂志封面的定性分析表明,封面代表是一个充分的机会,让美国医学会继续促进深色肤色病理意识。
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引用次数: 0
Severe nodulocystic acne requiring corticosteroids after social media-induced delay: a two-patient case series. 社交媒体导致的延迟后需要皮质类固醇的严重结节性痤疮:两例患者病例系列。
Q3 Medicine Pub Date : 2025-10-09 eCollection Date: 2025-12-01 DOI: 10.1093/skinhd/vzaf074
Nicolò Rivetti, Valeria Brazzelli, Carlo Francesco Tomasini, Stefania Barruscotti

Social media platforms, particularly TikTok, are increasingly used by adolescents for skincare advice. However, much of the content lacks medical accuracy and may delay appropriate treatment. We report two adolescent boys with severe nodulocystic acne who postponed dermatology care while following influencer-promoted routines. In both cases, the disease progressed to a highly inflammatory state, prompting the use of systemic corticosteroids before starting isotretinoin. The first patient, aged 16 years, presented with painful nodules after months of self-treatment; a short course of oral prednisone and antibiotics was followed by isotretinoin, with a good response. The second patient, aged 15 years, experienced similar progression and was also pretreated with oral steroids before isotretinoin initiation. Both achieved clinical improvement. These cases highlight the therapeutic consequences of digital misinformation and the role of corticosteroids as effective bridging therapy. They underscore the importance of early dermatology intervention and the need for evidence-based communication in online health spaces.

青少年越来越多地使用社交媒体平台,尤其是TikTok,寻求护肤建议。然而,许多内容缺乏医学准确性,可能会延误适当的治疗。我们报告了两名患有严重结节性痤疮的青春期男孩,他们推迟了皮肤科护理,而遵循影响者促进的常规。在这两种情况下,疾病进展到高度炎症状态,促使在开始使用异维甲酸之前使用全身皮质类固醇。第一位患者,16岁,自我治疗数月后出现疼痛结节;短期口服强的松和抗生素,随后异维甲酸,反应良好。第二名患者,15岁,经历了类似的进展,在异维甲酸开始前也接受了口服类固醇的预处理。两人均获得临床改善。这些病例强调了数字错误信息的治疗后果和皮质类固醇作为有效桥接治疗的作用。它们强调了早期皮肤病干预的重要性以及在在线卫生空间进行基于证据的交流的必要性。
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引用次数: 0
Epidemiology, clinical characteristics, flares and mortality of generalized pustular psoriasis: a nationwide register study in Finland. 广泛性脓疱性银屑病的流行病学、临床特征、发作和死亡率:芬兰的一项全国性登记研究。
Q3 Medicine Pub Date : 2025-10-09 eCollection Date: 2025-12-01 DOI: 10.1093/skinhd/vzaf076
Mirkka Koivusalo, Xavier Teitsma, Juha Mehtälä, Aino Vesikansa, Susanne Clemen Capion, Laura Airaksinen, Maria Grönman, Rafael Pasternack, Pauliina Nuutinen, Laura Huilaja

Background: Generalized pustular psoriasis (GPP) is a chronic and potentially life-threatening inflammatory disease characterized by recurrent, sudden flares often accompanied by systemic symptoms. With the introduction of new, targeted therapies for GPP there is an increased need to better understand patient characteristics to improve disease management.

Objectives: To assess the epidemiology of GPP, and compare clinical characteristics, treatments and overall survival between GPP and population-based controls and control participants with psoriasis vulgaris. Furthermore, flare occurrence and risk factors for flares were also assessed.

Methods: All Finnish patients with ≥1 GPP diagnosis (International Classification of Diseases, Tenth Revision: L40.1) in secondary healthcare between 1996 and 2021 and age- and sex-matched control groups were identified. Data on diagnoses, medications and deaths were collected from national registers. Incidence and prevalence were assessed using different case criteria. The primary analysis group included patients with ≥2 GPP diagnoses at a dermatology clinic in specialty care.

Results: GPP period prevalence decreased from 9.9 to 4.7 and the incidence rate from 4.2 to 2.1 per 100 000 when using stricter case criteria. Psoriasis (73%) and hypertension (49%) were the most common comorbidities among patients with GPP, and topical corticosteroids were the most used medication (92%; P < 0.05 vs. control groups). Overall survival was lower in GPP compared with both control groups (P < 0.001). In total, 43% of the patients experienced a flare during follow-up, of which one-third (33%) had a flare at the time of first GPP diagnosis. Systemic corticosteroid use [hazard ratio (HR) 1.41; 95% confidence interval (CI) 1.10-1.81; P = 0.007] was associated with a higher risk of GPP flares and flares were associated with an increased risk of death (HR 1.7; 95% CI 1.08-2.60; P < 0.05).

Conclusions: The epidemiology of GPP in Finland was found to be comparable to previously published estimates and associated with a high disease burden and shorter overall survival. Flares occurred frequently during follow-up and are associated with an increased risk of death. Standardized guidelines are crucial to improve the timely diagnosis and management of the disease.

背景:全身性脓疱性牛皮癣(GPP)是一种慢性和潜在威胁生命的炎症性疾病,其特征是反复发作,突然发作,常伴有全身症状。随着新的靶向治疗GPP的引入,更好地了解患者特征以改善疾病管理的需求增加。目的:评估GPP的流行病学,比较GPP与基于人群的对照组和普通银屑病对照组的临床特征、治疗方法和总生存率。此外,还对耀斑的发生和危险因素进行了评估。方法:选取1996年至2021年间在二级医疗机构中GPP诊断≥1(国际疾病分类,第十版:L40.1)的所有芬兰患者以及年龄和性别匹配的对照组。有关诊断、药物和死亡的数据是从国家登记册中收集的。发病率和流行率采用不同的病例标准进行评估。主要分析组包括在皮肤科诊所接受专科护理诊断的GPP≥2例的患者。结果:采用更严格的病例标准时,GPP期患病率从9.9降至4.7,发病率从4.2降至2.1 / 10万。银屑病(73%)和高血压(49%)是GPP患者最常见的合并症,外用皮质类固醇是使用最多的药物(92%,与对照组相比P < 0.05)。与对照组相比,GPP组的总生存率较低(P < 0.001)。总体而言,43%的患者在随访期间出现了耀斑,其中三分之一(33%)在首次GPP诊断时出现了耀斑。全身使用皮质类固醇[危险比(HR) 1.41;95%置信区间(CI) 1.10-1.81;P = 0.007]与GPP耀斑的高风险相关,而耀斑与死亡风险增加相关(HR 1.7; 95% CI 1.08-2.60; P < 0.05)。结论:芬兰GPP的流行病学发现与先前公布的估计相当,并与高疾病负担和较短的总生存期相关。在随访期间经常发生耀斑,并与死亡风险增加有关。标准化的指南对于提高疾病的及时诊断和管理至关重要。
{"title":"Epidemiology, clinical characteristics, flares and mortality of generalized pustular psoriasis: a nationwide register study in Finland.","authors":"Mirkka Koivusalo, Xavier Teitsma, Juha Mehtälä, Aino Vesikansa, Susanne Clemen Capion, Laura Airaksinen, Maria Grönman, Rafael Pasternack, Pauliina Nuutinen, Laura Huilaja","doi":"10.1093/skinhd/vzaf076","DOIUrl":"https://doi.org/10.1093/skinhd/vzaf076","url":null,"abstract":"<p><strong>Background: </strong>Generalized pustular psoriasis (GPP) is a chronic and potentially life-threatening inflammatory disease characterized by recurrent, sudden flares often accompanied by systemic symptoms. With the introduction of new, targeted therapies for GPP there is an increased need to better understand patient characteristics to improve disease management.</p><p><strong>Objectives: </strong>To assess the epidemiology of GPP, and compare clinical characteristics, treatments and overall survival between GPP and population-based controls and control participants with psoriasis vulgaris. Furthermore, flare occurrence and risk factors for flares were also assessed.</p><p><strong>Methods: </strong>All Finnish patients with ≥1 GPP diagnosis (International Classification of Diseases, Tenth Revision: L40.1) in secondary healthcare between 1996 and 2021 and age- and sex-matched control groups were identified. Data on diagnoses, medications and deaths were collected from national registers. Incidence and prevalence were assessed using different case criteria. The primary analysis group included patients with ≥2 GPP diagnoses at a dermatology clinic in specialty care.</p><p><strong>Results: </strong>GPP period prevalence decreased from 9.9 to 4.7 and the incidence rate from 4.2 to 2.1 per 100 000 when using stricter case criteria. Psoriasis (73%) and hypertension (49%) were the most common comorbidities among patients with GPP, and topical corticosteroids were the most used medication (92%; <i>P</i> < 0.05 vs. control groups). Overall survival was lower in GPP compared with both control groups (<i>P</i> < 0.001). In total, 43% of the patients experienced a flare during follow-up, of which one-third (33%) had a flare at the time of first GPP diagnosis. Systemic corticosteroid use [hazard ratio (HR) 1.41; 95% confidence interval (CI) 1.10-1.81; <i>P</i> = 0.007] was associated with a higher risk of GPP flares and flares were associated with an increased risk of death (HR 1.7; 95% CI 1.08-2.60; <i>P</i> < 0.05).</p><p><strong>Conclusions: </strong>The epidemiology of GPP in Finland was found to be comparable to previously published estimates and associated with a high disease burden and shorter overall survival. Flares occurred frequently during follow-up and are associated with an increased risk of death. Standardized guidelines are crucial to improve the timely diagnosis and management of the disease.</p>","PeriodicalId":74804,"journal":{"name":"Skin health and disease","volume":"5 6","pages":"432-442"},"PeriodicalIF":0.0,"publicationDate":"2025-10-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC12648532/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145643880","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}
引用次数: 0
期刊
Skin health and disease
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