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A generalized rash in an 8-year-old patient 一名 8 岁患者的全身皮疹
Pub Date : 2024-04-23 DOI: 10.1002/jvc2.422
Ashley S. Kim, Astrid H. Lossius

An 8-year-old patient was referred for a second opinion to our dermatology department from another dermatologist. The referral reported a 14-month history of therapy-resistant rash, originally treated as nummular atopic dermatitis. The patient reported spontaneous debut of an itchy rash to the extremities and trunk, with worsening of symptoms despite use of topical steroids and UVB light therapy. The rash spread gradually to the entire body, including face and scalp. Upon examination, there was a polymorphic presentation with well-demarcated red macules, small subcorneal pustules along borders, patchy hyperkeratosis and postinflammatory hyperpigmentation (Figure 1a,b). The patient also had patchy hair thinning with a ‘black dot pattern’ (Figure 2). This finding resulted in diagnostic workup to exclude fungal infection, subacute cutaneous lupus, psoriasis and secondary syphilis, in order of decreasing clinical likelihood. Skin and hair samples were both positive for dermatophyte DNA, Trichophyton species. Histology also reported fungal elements in the keratin layer. Treatment initiated with terbinafine 125 mg daily for 6 weeks, in addition to ketoconazole shampoo. Upon control, the patient presented with postinflammatory hyperpigmentation, however, her other symptoms had abated and she had experienced hair regrowth. Control skin samples were negative for dermatophyte DNA.

Ashley S. Kim wrote the manuscript and Astrid H. Lossius provided revisions. Both authors were involved in the workup and final diagnosis of this patient. Ashley S. Kim is the corresponding author.

The authors declare no conflict of interest.

The parents of minor patients have been given written informed consent for their child's participation in this article, as well as for the use of their child's deidentified, anonymized case details (including photographs) for publication. Ethical approval: Not applicable.

一位 8 岁的患者从另一位皮肤科医生处转诊到我院皮肤科寻求第二诊疗意见。转诊者称其皮疹病史长达14个月,最初被当作麻疹性特应性皮炎治疗。患者自发出现四肢和躯干瘙痒性皮疹,尽管使用了局部类固醇激素和紫外线照射疗法,但症状仍不断恶化。皮疹逐渐蔓延至全身,包括面部和头皮。经检查,患者的皮疹呈多形性,伴有边界清楚的红色斑丘疹、角质层下沿边界的小脓疱、斑片状角化过度和炎症后色素沉着(图 1a、b)。患者还伴有斑片状头发稀疏,呈 "黑点模式"(图 2)。诊断结果显示,按照临床可能性递减的顺序,患者可能患有真菌感染、亚急性皮肤狼疮、银屑病和继发性梅毒。皮肤和头发样本中的皮癣菌 DNA(毛癣菌)均呈阳性。组织学检查也显示角质层中有真菌成分。治疗开始时,除了使用酮康唑洗发水外,还每天使用特比萘芬 125 毫克,持续 6 周。治疗结束后,患者出现了炎症后色素沉着,但其他症状有所缓解,头发也重新生长。对照组皮肤样本中的皮癣菌DNA呈阴性。Ashley S. Kim撰写了手稿,Astrid H. Lossius提供了修改意见。两位作者都参与了该患者的检查和最终诊断。作者声明无利益冲突。未成年患者的父母已书面知情同意其子女参与本文的写作,并同意将其子女的去标识化、匿名化病例细节(包括照片)用于发表。伦理批准:不适用。
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引用次数: 0
Punch biopsies in tattooed skin: Complications and patient-reported outcomes 纹身皮肤的冲孔活检:并发症和患者报告结果
Pub Date : 2024-04-23 DOI: 10.1002/jvc2.431
Mila Poelhekken, Ines J. Schornagel, Sebastiaan A. S. van der Bent

Background

Despite the popularity and usage of tattoos in modern society, various complications can occur such as allergic tattoo reactions, infections, tattoo removal complications and chronic granulomatous inflammatory tattoo reactions such as sarcoidosis. Histopathology of skin biopsies in tattoos is crucial in the diagnostic process of complications. As tattoos are frequently obtained for cosmetic reasons, cosmetic consequences of a skin biopsy in a tattoo are important. Remarkably, no studies have been performed regarding the consequences and expected outcome of taking a punch biopsy of tattooed skin.

Objectives

The objective of this study is to report on the possible complications and patient-reported cosmetic outcomes after punch biopsies in tattooed skin. Specifically, this study aims to assess the impact on appearance, symptoms and psychosocial burden of biopsies in tattooed skin, using the SCAR-Q.

Methods

Patients who underwent a punch biopsy of tattooed skin at the Tattoo Clinic in the Alrijne Hospital between 2021 and 2023 were included. To evaluate patient satisfaction of the biopsy scar the SCAR-Q and a questionnaire regarding the recovery of the punch biopsy was used.

Results

The final cohort consisted of 50 patients. Across all scales, the median scores on the SCAR-Q questionnaire were consistent with the maximum score. Patients scored significantly better on the appearance scale when the biopsy was taken longer than 8 months ago. Two-third of the patients reported no postbiopsy symptoms, with the remainder experiencing itch, pain or redness, which typically resolved within 10 days (88%). Remarkably, 42% of the patients reported the biopsy scar was not visible on the skin anymore. Scars on the lower extremities and trunk were significantly more frequently visible, as well as scars among patients aged 40 and above.

Conclusions

This study demonstrates that the cosmetic and psychological impact of punch biopsies in tattooed skin is minimal and the recovery is fast with minimal symptoms. For this reason, biopsies of tattooed skin should not be avoided in the diagnostic process.

尽管纹身在现代社会很流行,使用率也很高,但仍可能出现各种并发症,如过敏性纹身反应、感染、纹身去除并发症和慢性肉芽肿炎症性纹身反应(如肉瘤病)。纹身皮肤活检组织病理学是诊断并发症的关键。由于纹身通常是为了美观,因此在纹身处进行皮肤活检对美观的影响非常重要。本研究的目的是报告纹身皮肤打孔活检后可能出现的并发症和患者报告的美容效果。具体而言,本研究旨在使用 SCAR-Q 评估纹身活检对外观、症状和社会心理负担的影响。研究对象包括 2021 年至 2023 年期间在阿尔里内医院纹身诊所接受纹身活检的患者。为了评估患者对活检疤痕的满意度,我们使用了 SCAR-Q 和一份有关打孔活检恢复情况的问卷。在所有量表中,SCAR-Q 问卷的中位数得分与最高分一致。当活检时间超过 8 个月时,患者的外观评分明显较高。三分之二的患者表示活检后没有任何症状,其余患者有痒、痛或发红的症状,这些症状通常在 10 天内缓解(88%)。值得注意的是,42% 的患者表示活检疤痕在皮肤上已经看不到了。下肢和躯干的疤痕明显更常见,40 岁及以上患者的疤痕也更明显。这项研究表明,在有纹身的皮肤上进行打孔活检对美容和心理的影响很小,而且恢复快,症状轻微。因此,在诊断过程中不应避免对纹身皮肤进行活检。
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引用次数: 0
Blaschkolinear comedonal nodules and plaques with congenital onset 伴有先天性发病的布拉什科利奈角瘤样结节和斑块
Pub Date : 2024-04-19 DOI: 10.1002/jvc2.420
Kaan Yilmaz, Leonie Rabe, Cyrill Géraud, Marthe-Lisa Schaarschmidt
<p>A 27-year-old Caucasian male presented with disseminated linear patches of comedones in the head and neck region, on the trunk and extremities. They were readily apparent at birth and have gradually progressed in size whilst being extended by indolent, large nodulocystic lesions during adolescence (Figure 1). He had an unremarkable family history of skin disease and did not report any preexisting conditions. Notably, the patient underwent an excision of a mandibular tumour at the age of 14, which was histologically difficult to classify, but ultimately found to be suggestive of a giant cell granuloma in association with a follicular cyst. No systemic abnormalities, including any visual, skeletal or neurologic impairment, were reported.</p><p>On physical examination, extensive curvilinear, bilateral, mostly alopecic patches of densely distributed open comedones were observed in a Blaschkoid pattern on the face, scalp, neck, trunk and proximal portions of the upper extremities. The scalp and the back were further studded with multiple noninflammatory, partially coalescent cystic nodules reaching up to 6 cm in diameter (Figures 1 and 2). Two skin biopsies were taken from the cystic areas on the back and the occipital region, respectively (Figure 3).</p><p>Naevus comedonicus (NC).</p><p>The histopathologic assessment of the biopsy specimens revealed epidermoid cysts comprising regular stratified squamous epithelium with stratum granulosum and lamellar orthokeratosis (Figure 3). Next-generation sequencing of another skin biopsy specimen from the back yielded the in-frame deletion variant c.1755_1757del p.(Thr586del) in the <i>Never in Mitosis Gene A-Related Kinase 9</i> (<i>NEK9</i>) gene with a mosaic frequency of 30%. In contrast, no pathogenic <i>NEK9</i> variants were identified in DNA extracted from EDTA blood of the patient, indicating a somatic variant in the <i>NEK9</i> gene, which is the molecular aetiology of NC.</p><p>Subsequently, the patient was thoroughly examined for extracutaneous manifestations. In this regard, ophthalmologic evaluation revealed, apart from myopia and astigmatism, no other abnormalities, particularly no cataracts. The echocardiographic assessment showed an annuloaortic ectasia as well as a trivial mitral and tricuspid valve regurgitation, albeit without relevant congenital cardiovascular malformations. Cranial magnetic resonance imaging and computed tomography scan detected no structural changes. No neurologic and skeletal anomalies were found.</p><p>NC (MIM #617025) is a rare organoid epidermal naevus characterized by a hereditary malformation of the pilosebaceous unit, leading to an abrogated production of terminal hair and sebaceous glands.<span><sup>1, 2</sup></span> The histopathologic correlate is the plugging of dilated follicular ostia by lamellar keratinaceous material, which clinically manifests as comedones in a honeycomb pattern, mostly commencing in infancy.<span><sup>3</sup></span> While NC predominan
一名 27 岁的白种男性头颈部、躯干和四肢出现散在的线状粉刺斑块。这些粉刺在出生时就很明显,在青春期时逐渐增大,并扩展为不活跃的大结节性囊肿病变(图 1)。他没有明显的皮肤病家族史,也没有报告任何原有疾病。值得注意的是,患者在14岁时接受了下颌骨肿瘤切除术,该肿瘤在组织学上很难分类,但最终发现提示为巨细胞肉芽肿合并滤泡囊肿。体格检查时,在患者的面部、头皮、颈部、躯干和上肢近端发现了广泛的双侧弧形、多为脱发的斑块,密集分布的开放性粉刺呈布拉什科德(Blaschkoid)型。头皮和背部还出现了多个非炎症性、部分凝聚的囊性结节,直径可达 6 厘米(图 1 和图 2)。活检标本的组织病理学评估显示,表皮样囊肿由规则的分层鳞状上皮与颗粒层和片状角化层组成(图 3)。另一份背部皮肤活检标本的下一代测序结果显示,NEK9(Never in Mitosis Gene A-Related Kinase 9)基因中的c.1755_1757del p.(Thr586del)框内缺失变异,嵌套频率为30%。相比之下,从患者 EDTA 血液中提取的 DNA 未发现 NEK9 致病变体,这表明 NEK9 基因存在体细胞变异,而这正是 NC 的分子病因。在这方面,眼科评估显示,除了近视和散光外,没有其他异常,尤其是没有白内障。超声心动图评估显示环状主动脉异位以及轻微的二尖瓣和三尖瓣反流,但没有相关的先天性心血管畸形。头颅磁共振成像和计算机断层扫描未发现结构性变化。NC(MIM #617025)是一种罕见的器质性表皮痣,其特征是毛囊单位的遗传性畸形,导致末端毛发和皮脂腺的生成减弱、2 与之相关的组织病理学特征是片状角质物质堵塞扩张的毛囊口,临床表现为蜂窝状的粉刺,大多在婴儿期发病。虽然 NC 主要发生在单侧,但据报道,像我们患者一样双侧受累的病例约占 35%。最近描述了 NC 的三种临床表型,即:(i) 以粉刺型为主;(ii) 伴有巨大粉刺、囊肿和结节并伴有炎症和瘢痕形成的 "Selhorst 型";(iii) 伴有粉刺的萎缩性疣样点状萎缩性疤痕。在这种情况下,就出现了一个问题,即所提出的亚型是代表不同的形态变异,还是属于具有波动和表型转变的疾病连续体的一部分,这有待于今后的研究。另一方面,重要的是要区分 NC 和其他形式的器质性和非器质性(角质细胞)表皮痣,它们可能同样表现为广泛的 Blaschkolinear 模式和/或作为孪生斑点现象(didymosis)并存。5、6 NC 和皮肤外异常的组合也被称为黑痣综合征(NCS)。7 尽管缺乏明确的共识诊断标准,但它主要与眼部、骨骼和神经系统异常有关,而同侧白内障被认为是 NCS 最特殊的特征、6、7 在我们的患者中,没有发现综合征的充分证据,但下颌骨囊性肿瘤是否可能代表一种迄今未报道的表现仍令人难以捉摸。Blaschkoid分布可归因于NEK9基因的致病性杂合子后嵌合,该基因被确定为NC和NCS的致病突变。迄今为止,已报道的 NEK9 基因变异不到 10 个,所有变异均局限于其染色体凝集样调节器(RCC1)和激酶结构域。8-10 有趣的是,在我们的患者中发现的 NEK9 基因 RCC1 结构域中的 p.(Thr586del) 框内缺失,迄今为止仅在另外两名 NC 和/或 NCS 患者中观察到。
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引用次数: 0
Assessment of atopic dermatitis in all phototypes using line-field confocal optical coherence tomography (LC-OCT) and reflectance confocal microscopy (RCM) 使用线场共聚焦光学相干断层扫描(LC-OCT)和反射共聚焦显微镜(RCM)评估所有光型的特应性皮炎
Pub Date : 2024-04-16 DOI: 10.1002/jvc2.421
Samantha Ouellette, Thu Minh Truong, Samavia Khan, Sara Ragi, Shazli Razi, Babar Rao

Background

Theclinical presentation of atopic dermatitis (AD) varies in different skin phototypes and has been evaluated previously using reflectance confocal microscopy (RCM). Line-field confocal optical coherence tomography (LC-OCT) is a new noninvasive imaging modality with additional capabilities.

Objectives

This prospective cohort study aimed to assess the capabilities of LC-OCT and RCM in visualizing and quantifying microscopic features of AD lesions.

Methods

Ten patients with clinically diagnosed AD and visible active lesions were included in the study. LC-OCT and RCM imaging were performed on lesional and perilesional skin during routine dermatology visits. Image analysis included quantitative measurements of living and total epidermal thickness and dermo-epidermal junction (DEJ) undulation using LC-OCT, as well as visual assessment of characteristic features such as spongiosis, exocytosis, perivascular inflammation, and atypical blood vessels using both LC-OCT and RCM.

Results

LC-OCT provided precise measurements of epidermal landmarks and shared similar microscopic features compared to RCM. AD lesions exhibited increased stratum corneum thickness, living epidermis thickness, total epidermis thickness, and DEJ undulation compared to clinically normal skin. Spongiosis, exocytosis, perivascular inflammation, blood vessel proliferation, and dilation were prominent features observed in AD lesions on both LC-OCT and RCM images. Interestingly, some inflammatory features were also detected in the visually normal skin of AD patients.

Conclusions

LC-OCT showed potential as a fast and noninvasive imaging tool for assessing inflammatory skin pathologies, including AD. Its ability to visualize microscopic features and quantify epidermal changes may aid in diagnosis and treatment monitoring. These findings suggest that LC-OCT could provide valuable insights, particularly for patients with darker skin types where clinical severity assessment may be challenging. Further studies are warranted to explore the full potential of LC-OCT and its integration into clinical practice.

特应性皮炎(AD)在不同皮肤光型中的临床表现各不相同,以前曾使用反射共聚焦显微镜(RCM)对其进行过评估。这项前瞻性队列研究旨在评估 LC-OCT 和 RCM 在观察和量化 AD 病变微观特征方面的能力。在常规皮肤科就诊期间,对病变和周围皮肤进行了 LC-OCT 和 RCM 成像检查。图像分析包括使用 LC-OCT 定量测量活表皮和总表皮厚度以及真皮-表皮交界处(DEJ)的起伏,以及使用 LC-OCT 和 RCM 对海绵化、外渗、血管周围炎症和非典型血管等特征进行视觉评估。与临床正常皮肤相比,AD 病变表现为角质层厚度、活表皮厚度、表皮总厚度和 DEJ 起伏增加。在 LC-OCT 和 RCM 图像上观察到的突出特征包括海绵化、外渗、血管周围炎症、血管增生和扩张。LC-OCT作为一种快速、无创的成像工具,在评估包括AD在内的炎症性皮肤病变方面显示出了潜力。LC-OCT作为一种快速、无创的成像工具,在评估包括AD在内的炎症性皮肤病变方面显示出了潜力,其可视化微观特征和量化表皮变化的能力可能有助于诊断和治疗监测。这些研究结果表明,LC-OCT 可以提供有价值的见解,特别是对于临床严重程度评估可能具有挑战性的深色皮肤类型患者。还需要进一步的研究来探索 LC-OCT 的全部潜力,并将其融入临床实践。
{"title":"Assessment of atopic dermatitis in all phototypes using line-field confocal optical coherence tomography (LC-OCT) and reflectance confocal microscopy (RCM)","authors":"Samantha Ouellette,&nbsp;Thu Minh Truong,&nbsp;Samavia Khan,&nbsp;Sara Ragi,&nbsp;Shazli Razi,&nbsp;Babar Rao","doi":"10.1002/jvc2.421","DOIUrl":"10.1002/jvc2.421","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Background</h3>\u0000 \u0000 <p>Theclinical presentation of atopic dermatitis (AD) varies in different skin phototypes and has been evaluated previously using reflectance confocal microscopy (RCM). Line-field confocal optical coherence tomography (LC-OCT) is a new noninvasive imaging modality with additional capabilities.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Objectives</h3>\u0000 \u0000 <p>This prospective cohort study aimed to assess the capabilities of LC-OCT and RCM in visualizing and quantifying microscopic features of AD lesions.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>Ten patients with clinically diagnosed AD and visible active lesions were included in the study. LC-OCT and RCM imaging were performed on lesional and perilesional skin during routine dermatology visits. Image analysis included quantitative measurements of living and total epidermal thickness and dermo-epidermal junction (DEJ) undulation using LC-OCT, as well as visual assessment of characteristic features such as spongiosis, exocytosis, perivascular inflammation, and atypical blood vessels using both LC-OCT and RCM.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>LC-OCT provided precise measurements of epidermal landmarks and shared similar microscopic features compared to RCM. AD lesions exhibited increased stratum corneum thickness, living epidermis thickness, total epidermis thickness, and DEJ undulation compared to clinically normal skin. Spongiosis, exocytosis, perivascular inflammation, blood vessel proliferation, and dilation were prominent features observed in AD lesions on both LC-OCT and RCM images. Interestingly, some inflammatory features were also detected in the visually normal skin of AD patients.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusions</h3>\u0000 \u0000 <p>LC-OCT showed potential as a fast and noninvasive imaging tool for assessing inflammatory skin pathologies, including AD. Its ability to visualize microscopic features and quantify epidermal changes may aid in diagnosis and treatment monitoring. These findings suggest that LC-OCT could provide valuable insights, particularly for patients with darker skin types where clinical severity assessment may be challenging. Further studies are warranted to explore the full potential of LC-OCT and its integration into clinical practice.</p>\u0000 </section>\u0000 </div>","PeriodicalId":94325,"journal":{"name":"JEADV clinical practice","volume":"3 3","pages":"817-826"},"PeriodicalIF":0.0,"publicationDate":"2024-04-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1002/jvc2.421","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140696281","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
Ultra-low dose radiation therapy for primary cutaneous indolent B-cell lymphomas 超低剂量放射治疗原发性皮肤轻度 B 细胞淋巴瘤
Pub Date : 2024-04-15 DOI: 10.1002/jvc2.403
Shalini Kasera, Christopher R. Weil, David Wada, Marianne Bowling, Boyu Hu, David K. Gaffney, Amit Maity, Randa Tao

Background

Primary cutaneous follicle centre lymphoma (PCFCL) and primary cutaneous marginal zone lymphoma (PCMZL) are indolent radiosensitive lymphomas, with national guidelines recommending 24 Grey (Gy) in 12 fractions, despite shorter regimens showing no difference in overall survival. Multiple studies demonstrate 4 Gy in two fractions is effective in other indolent lymphomas. Shorter regimens may result in less toxicity for patients.

Objectives

We aim to characterise local control rates in PCFCL and PCMZL treated with radiation therapy (RT) with 4 Gy in two fractions.

Methods

From a single institution database, we retrospectively identified all patients with PCFCL and PCMZL treated with 4 Gy in two fractions between 2011 and 2022. Prior topical or systemic treatments were permitted. T-cell or high-grade B-cell cutaneous lymphomas, or those with less than 6 months follow-up, were excluded. Primary outcomes were rates of response and local failure per lesion as determined by a radiation oncologist and oncologic dermatologist.

Results

Forty-three consecutive patients with 98 lesions treated were identified. Twenty-four (56%) of the patients had PCMZL, and the remainder had PCFCL. Most lesions (97%) received RT with electrons. The complete response or partial response rate was 94%. Six local failures were identified (6%). With a median follow-up of 2.75 years, 1-, 2- and 3-year local failure-free survival were 94%, 94% and 92%, respectively. There was minimal acute toxicity, no late toxicity and no cosmesis issues. No clinical features were predictive of local failure.

Conclusions

Ultra-low dose radiation was associated with high rates of local control and minimal toxicity and may reduce personal burden and financial toxicity in appropriately selected patients.

原发性皮肤滤泡中心淋巴瘤(PCFCL)和原发性皮肤边缘区淋巴瘤(PCMZL)是对放射线敏感的惰性淋巴瘤,国家指南建议采用 24 Grey(Gy),12 次分次治疗,尽管较短的治疗方案显示总生存率没有差异。多项研究表明,对其他惰性淋巴瘤来说,4Gy、2 次分次放疗是有效的。我们从单个机构的数据库中,回顾性地发现了2011年至2022年间所有接受4 Gy两分次放射治疗的PCFCL和PCMZL患者。允许之前接受过局部或全身治疗。T细胞或高级别B细胞皮肤淋巴瘤或随访时间少于6个月的患者除外。主要结果是由放射肿瘤专家和肿瘤皮肤科专家确定的每个病灶的反应率和局部失败率。其中 24 例(56%)患者患有 PCMZL,其余为 PCFCL。大多数病灶(97%)接受了电子 RT 治疗。完全应答或部分应答率为 94%。共发现 6 例局部失败病例(6%)。中位随访时间为2.75年,1年、2年和3年无局部失败生存率分别为94%、94%和92%。急性毒性极低,无晚期毒性,无外观问题。超低剂量放射治疗的局部控制率高、毒性小,经适当选择的患者可减少个人负担和经济损失。
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引用次数: 0
Pityriasis rosea presenting with a heraldic patch within a tattoo 纹身处出现纹章斑块的玫瑰糠疹
Pub Date : 2024-04-13 DOI: 10.1002/jvc2.417
Nicolas Kluger, Lidiya N. Todorova

Pityriasis rosea (PR) is an acute, self-healing exanthema, affecting mainly young patients, that can disclose various clinical presentation.1 The elective location to traumatised areas has not been reported during PR.

A 21-year-old otherwise healthy woman presented with small rose scaly ovoid patches of the trunk (Figure 1). She reported having profuse rhinorrhea 2–3 weeks before the appearance of the rash and no other symptoms. She recalled an initial larger patch within a thin black tattoo of the left side of the trunk (Figure 2) before the rash spread to the rest of the body. A diagnosis of PR was made.

Except Koebner phenomenon—prone diseases like psoriasis, lichen or vitiligo, many dermatoses have been anecdotally described in tattoos.2 PR is not known to display affinity to traumatised areas1, 3 and, to our knowledge, PR has never been reported within tattoos.4 The elective localisation to a tattoo in our case is most likely merely fortuitous, but we cannot rule out that previous cases might have gone unnoticed or been unreported. The possible occurrence of a herald patch in a tattoo is to be kept in mind as it could be mistaken initially for psoriasis or tinea corporis5

Lidiya N. Todorova contributed to the collection of data and management of the patient. Nicolas Kluger wrote the first draft of the manuscript. Lidiya N. Todorova and Nicolas Kluger revised the manuscript and prepared the final version. All authors have read and approved the final manuscript and agree to take full responsibility for the integrity and accuracy of the work.

The authors declare no conflicts of interest.

The patient in this manuscript has given written informed consent for participation in the study and the use of their deidentified, anonymized, aggregated data and their case details (including photographs) for publication. Ethical Approval: not applicable.

玫瑰糠疹(PR)是一种急性、自愈性外皮癣,主要影响年轻患者,临床表现多种多样1 。她说在皮疹出现前 2-3 周曾出现大量鼻出血,但没有其他症状。她回忆说,在皮疹扩散到身体其他部位之前,躯干左侧的黑色细纹内最初出现过一块较大的斑块(图 2)。除了银屑病、苔癣或白癜风等易发生柯布纳现象的疾病外,许多皮肤病都曾在纹身上出现过轶事描述2 。据我们所知,PR 与创伤部位没有亲缘关系1、3 ,而且从未有在纹身上出现 PR 的报道4 。需要注意的是,纹身中可能会出现预示斑,因为最初可能会被误认为是银屑病或体癣5。Nicolas Kluger 撰写了手稿初稿。Lidiya N. Todorova 和 Nicolas Kluger 对手稿进行了修改,并编写了最终版本。所有作者均已阅读并批准最终稿件,并同意对作品的完整性和准确性承担全部责任。作者声明无利益冲突。本稿件中的患者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细节(包括照片)用于发表。伦理批准:不适用。
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引用次数: 0
Generalised exanthematous pustular dermatophytids during Trichophyton violaceum tinea capitis 毛癣菌性头癣期间的全身性泛发性脓疱性皮炎
Pub Date : 2024-04-13 DOI: 10.1002/jvc2.416
Nicolas Kluger

Dermatophytids are defined as the acute occurrence of noninfectious secondary lesions at distance from a primary dermatophytic infection. They are well-known after intertrigo of the toes. We report an 8-year-old boy, who developed generalised pustular lesions during the treatment of tinea capitis caused by Trichophyton violaceum. He had a rapid response to local corticosteroid ointment, while oral terbinafine was efficient on the scalp condition. Dermatophytids are the result of a type IV delayed hypersensitivity reaction to dermatophytic antigens. They may occur after initiation of an antifungal treatment. Generalised exanthematous pustular dermatophytids is a clinical variant of dermatophytids. They present as nonfollicular, nonconfluent pustules with an erythematous base, of abrupt onset. Main differential diagnoses are acute generalised exanthematous pustulosis and pustular psoriasis. Medical history, clinical presentation, laboratory findings and, if necessary, biopsy will help to distinguish between those conditions. Evolution is rapidly favourable under local or oral corticotherapy and antifungal treatments should not be withdrawn. Dermatophytids are a differential diagnostic of acute generalised pustulosis during tinea capitis.

皮癣是指在原发皮癣感染的基础上急性发生的非感染性继发病变。它们是继足趾间皮瘤之后众所周知的疾病。我们报告了一名 8 岁男孩,他在治疗由毛癣菌引起的头癣期间出现了全身性脓疱病。他对局部皮质类固醇软膏反应迅速,而口服特比萘芬对头皮病症有效。皮癣是对皮癣抗原的 IV 型迟发性超敏反应的结果。它们可能在开始抗真菌治疗后发生。全身泛发性脓疱型皮癣是皮癣的一种临床变异型。它们表现为非叶状、非融合性脓疱,基底红斑,发病突然。主要鉴别诊断为急性泛发性脓疱病和脓疱性银屑病。病史、临床表现、实验室检查结果以及必要时的活组织检查有助于区分这些疾病。在局部或口服皮质类固醇激素治疗后,病情会迅速好转,但不应停止抗真菌治疗。皮肤癣菌是头癣急性全身脓疱病的鉴别诊断依据。
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引用次数: 0
VEXAS syndrome: Focus on dermatological manifestations and their histopathological correlate VEXAS 综合征:关注皮肤病表现及其组织病理学相关性
Pub Date : 2024-04-11 DOI: 10.1002/jvc2.395
Sofie Engelen, Anne-Catherine Dens, Frederik Staels, Rik Schrijvers, Daniel Blockmans, Steven Vanderschueren, Albrecht Betrains, F. J. Sherida H. Woei-A-Jin, Arno Vanstapel, Franscesca Bosisio, Petra De Haes

Background

VEXAS ‘Vacuoles, E1 Enzyme, X-linked, Autoinflammatory, Somatic syndrome’ is a rare autoinflammatory syndrome, first described in October 2020 by Beck et al. It is caused by somatic mutations in the UBA1 gene, coding for the E1 enzyme, responsible for ubiquitination. It manifests in adulthood, mainly in men, with constitutional symptoms, haematological abnormalities and often skin lesions with neutrophilic dermatoses most frequently reported. Since skin lesions are a frequent and early manifestation, recognising these may prove very useful for diagnosis.

Objectives

To focus on the dermatological manifestations of VEXAS and their histopathological correlate.

Methods

We retrospectively collected data and revised skin biopsies of VEXAS patients diagnosed in our tertiary care centre, and compared those with the literature.

Results

We identified nine men between 62 and 84 years old. The most frequently encountered UBA1 mutation was p.Met41Thr. Skin manifestations occurred in all patients; mostly as erythematous to purpuric papules/plaques, often with annular pattern. Histopathological, early VEXAS lesions showed a gradient in infiltrate with neutrophils being concentrated in the superficial interstitium and lymphocytes situated deeper around the blood vessels. Later in the disease course, the pattern became more variable and less specific with more nuclear debris, histiocytes, neutrophils and ulceration. Haematological and constitutional manifestations were present in all patients, followed by musculoskeletal (88.8%), eye (77.7%) and cardiovascular manifestations (66.6%). Polychondritis was present in 6/9 (66.6%) patients, as were respiratory symptoms, though only 33.3% had proven lung disease. The mortality rate was 22% 10 months after diagnosis.

Conclusions

This is the first cohort which describes in detail VEXAS skin manifestations with revision of all available skin biopsies, which led to the conclusion that early histopathological abnormalities in VEXAS syndrome may be easy to recognise, while the histopathological image becomes less specific over time and mimics other diseases as VEXAS progresses.

VEXAS "空泡、E1 酶、X 连锁、自身炎症、体细胞综合征 "是一种罕见的自身炎症综合征,由 Beck 等人于 2020 年 10 月首次描述。它是由负责泛素化的 E1 酶编码的 UBA1 基因体细胞突变引起的。该病在成年后发病,主要是男性,表现为全身症状、血液学异常和皮肤病变,其中以嗜中性粒细胞皮肤病最为常见。我们回顾性地收集了在我们的三级医疗中心确诊的 VEXAS 患者的数据和皮肤活检结果,并与文献进行了比较。最常见的 UBA1 基因突变是 p.Met41Thr。所有患者均有皮肤表现,大多为红斑至紫癜性丘疹/斑块,常呈环状。组织病理学显示,VEXAS 早期病变呈梯度浸润,中性粒细胞集中在表层间质,而淋巴细胞则位于血管周围的深层。病程后期,浸润模式变得更加多变,特异性降低,出现更多的核碎屑、组织细胞、中性粒细胞和溃疡。所有患者都有血液和体征表现,其次是肌肉骨骼(88.8%)、眼睛(77.7%)和心血管(66.6%)表现。6/9(66.6%)名患者出现了多软骨炎,呼吸道症状也出现了多软骨炎,但只有 33.3% 的患者被证实患有肺部疾病。这是首个详细描述VEXAS皮肤表现的队列,对所有可用的皮肤活检进行了修订,从而得出结论:VEXAS综合征的早期组织病理学异常可能很容易识别,而随着时间的推移,组织病理学图像的特异性会降低,并随着VEXAS的进展而模仿其他疾病。
{"title":"VEXAS syndrome: Focus on dermatological manifestations and their histopathological correlate","authors":"Sofie Engelen,&nbsp;Anne-Catherine Dens,&nbsp;Frederik Staels,&nbsp;Rik Schrijvers,&nbsp;Daniel Blockmans,&nbsp;Steven Vanderschueren,&nbsp;Albrecht Betrains,&nbsp;F. J. Sherida H. Woei-A-Jin,&nbsp;Arno Vanstapel,&nbsp;Franscesca Bosisio,&nbsp;Petra De Haes","doi":"10.1002/jvc2.395","DOIUrl":"10.1002/jvc2.395","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Background</h3>\u0000 \u0000 <p>VEXAS ‘Vacuoles, E1 Enzyme, X-linked, Autoinflammatory, Somatic syndrome’ is a rare autoinflammatory syndrome, first described in October 2020 by Beck et al. It is caused by somatic mutations in the <i>UBA1</i> gene, coding for the E1 enzyme, responsible for ubiquitination. It manifests in adulthood, mainly in men, with constitutional symptoms, haematological abnormalities and often skin lesions with neutrophilic dermatoses most frequently reported. Since skin lesions are a frequent and early manifestation, recognising these may prove very useful for diagnosis.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Objectives</h3>\u0000 \u0000 <p>To focus on the dermatological manifestations of VEXAS and their histopathological correlate.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>We retrospectively collected data and revised skin biopsies of VEXAS patients diagnosed in our tertiary care centre, and compared those with the literature.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>We identified nine men between 62 and 84 years old. The most frequently encountered <i>UBA1</i> mutation was p.Met41Thr. Skin manifestations occurred in all patients; mostly as erythematous to purpuric papules/plaques, often with annular pattern. Histopathological, early VEXAS lesions showed a gradient in infiltrate with neutrophils being concentrated in the superficial interstitium and lymphocytes situated deeper around the blood vessels. Later in the disease course, the pattern became more variable and less specific with more nuclear debris, histiocytes, neutrophils and ulceration. Haematological and constitutional manifestations were present in all patients, followed by musculoskeletal (88.8%), eye (77.7%) and cardiovascular manifestations (66.6%). Polychondritis was present in 6/9 (66.6%) patients, as were respiratory symptoms, though only 33.3% had proven lung disease. The mortality rate was 22% 10 months after diagnosis.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusions</h3>\u0000 \u0000 <p>This is the first cohort which describes in detail VEXAS skin manifestations with revision of all available skin biopsies, which led to the conclusion that early histopathological abnormalities in VEXAS syndrome may be easy to recognise, while the histopathological image becomes less specific over time and mimics other diseases as VEXAS progresses.</p>\u0000 </section>\u0000 </div>","PeriodicalId":94325,"journal":{"name":"JEADV clinical practice","volume":"3 4","pages":"1035-1048"},"PeriodicalIF":0.0,"publicationDate":"2024-04-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1002/jvc2.395","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140713003","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
Necrobiotic xanthogranuloma: A case report of successful treatment with intravenous immunoglobulins 坏死性黄疽瘤:静脉注射免疫球蛋白成功治疗的病例报告
Pub Date : 2024-04-11 DOI: 10.1002/jvc2.411
Julia K. Winkler, Holger A. Haenssle, Jochen Hoffmann, Ferdinand Toberer, Alexander H. Enk

Necrobiotic xanthogranuloma (NXG) is a rare non-Langerhans cell histiocytosis associated with paraproteinemia. Skin lesions of NXG are difficult to treat and various therapies have been evaluated with inconsistent results. A therapeutic standard has not been established. We report on a patient severely affected by mutilating skin and ocular lesions as well as lung infiltrates of necrobiotic xanthogranuloma. After failure of several previous therapies including dapsone and adalimumab, lesions significantly improved during therapy with intravenous immunoglobulins (IVIg). IVIg are an effective treatment option to evaluate in NXG.

坏死性黄疽瘤(NXG)是一种罕见的非朗格汉斯细胞组织细胞增生症,伴有副蛋白血症。NXG 皮肤病变很难治疗,对各种疗法进行了评估,但结果并不一致。治疗标准尚未确立。我们报告了一名因皮肤和眼部损伤以及肺部坏死性黄疽浸润而受到严重影响的患者。在之前的几种疗法(包括达泊松和阿达木单抗)失败后,患者在接受静脉注射免疫球蛋白(IVIg)治疗期间,皮损得到了明显改善。IVIg是评估NXG的一种有效治疗方案。
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引用次数: 0
Lip necrosis caused by methicillin-resistant Staphylococcus aureus (MRSA) 耐甲氧西林金黄色葡萄球菌(MRSA)引起的唇坏死
Pub Date : 2024-04-09 DOI: 10.1002/jvc2.418
Saad Khayat, Marc Agea Martínez, Manuel Tousidonis Rial, Alberto Diez Montiel, Santiago Ochandiano Caicoya

Labial necrosis is a rare condition with diverse causes, posing challenges for diagnosis and treatment. Methicillin-resistant Staphylococcus aureus (MRSA) strains, particularly those positive for Panton-Valentine leukocidin (PVL), further complicate management due to antibiotic resistance and heightened virulence. We present three cases of labial necrosis caused by MRSA, two of which resulted positive for PVL. The first case involved a 35-year-old male with persistent lip edema and inflammation, which progressed despite various treatments. MRSA was isolated, and the patient responded to daptomycin and clindamycin therapy. The second case featured a 38-year-old male with intense lip pain and swelling, initially misdiagnosed as angioedema. The patient improved after treatment with daptomycin and clindamycin, along with nasal decolonization. The third case involved a 19-year-old male with rapid upper lip swelling following manipulation of a boil. Prompt drainage and antibiotic treatment led to complete resolution. These cases underscore the importance of considering MRSA and PVL-positive S. aureus as potential etiologies in labial necrosis, especially in young individuals without underlying medical conditions. Effective management involves incision and drainage, complemented by antibiotic therapy tailored to drug resistance and virulence factors.

唇坏死是一种罕见病,病因多种多样,给诊断和治疗带来了挑战。耐甲氧西林金黄色葡萄球菌(MRSA)菌株,尤其是对潘顿-瓦伦丁白细胞介素(PVL)呈阳性的菌株,由于抗生素耐药性和毒力增强,使治疗更加复杂。我们介绍了三例由 MRSA 引起的唇坏死病例,其中两例的 PVL 检测结果呈阳性。第一例患者是一名 35 岁的男性,嘴唇持续水肿和发炎,尽管采取了各种治疗措施,但情况仍在恶化。分离出 MRSA,患者对达托霉素和克林霉素治疗有反应。第二个病例是一名 38 岁的男性,嘴唇剧烈疼痛和肿胀,最初被误诊为血管性水肿。患者在接受达托霉素和克林霉素治疗以及鼻腔脱落细胞治疗后病情有所好转。第三个病例涉及一名 19 岁的男性,在操作疖子后上唇迅速肿胀。经过及时的引流和抗生素治疗后,患者的病情得到了完全缓解。这些病例强调了将 MRSA 和 PVL 阳性金黄色葡萄球菌视为唇坏死潜在病因的重要性,尤其是对于没有潜在疾病的年轻人。有效的治疗方法包括切开引流,并根据耐药性和毒力因素辅以抗生素治疗。
{"title":"Lip necrosis caused by methicillin-resistant Staphylococcus aureus (MRSA)","authors":"Saad Khayat,&nbsp;Marc Agea Martínez,&nbsp;Manuel Tousidonis Rial,&nbsp;Alberto Diez Montiel,&nbsp;Santiago Ochandiano Caicoya","doi":"10.1002/jvc2.418","DOIUrl":"10.1002/jvc2.418","url":null,"abstract":"<p>Labial necrosis is a rare condition with diverse causes, posing challenges for diagnosis and treatment. Methicillin-resistant <i>Staphylococcus aureus</i> (MRSA) strains, particularly those positive for Panton-Valentine leukocidin (PVL), further complicate management due to antibiotic resistance and heightened virulence. We present three cases of labial necrosis caused by MRSA, two of which resulted positive for PVL. The first case involved a 35-year-old male with persistent lip edema and inflammation, which progressed despite various treatments. MRSA was isolated, and the patient responded to daptomycin and clindamycin therapy. The second case featured a 38-year-old male with intense lip pain and swelling, initially misdiagnosed as angioedema. The patient improved after treatment with daptomycin and clindamycin, along with nasal decolonization. The third case involved a 19-year-old male with rapid upper lip swelling following manipulation of a boil. Prompt drainage and antibiotic treatment led to complete resolution. These cases underscore the importance of considering MRSA and PVL-positive <i>S. aureus</i> as potential etiologies in labial necrosis, especially in young individuals without underlying medical conditions. Effective management involves incision and drainage, complemented by antibiotic therapy tailored to drug resistance and virulence factors.</p>","PeriodicalId":94325,"journal":{"name":"JEADV clinical practice","volume":"3 4","pages":"1253-1257"},"PeriodicalIF":0.0,"publicationDate":"2024-04-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1002/jvc2.418","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140723372","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
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JEADV clinical practice
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