cutaneous manifestations

皮肤表现
  • 文章类型: Case Reports
    PSTPIP1相关的骨髓相关蛋白血症炎性(PAMI)综合征是一种罕见的自身炎症性疾病,通常在儿科患者中出现。我们介绍了一个18岁女性的病例,有生长失败的病史,免疫球蛋白A肾病,和炎症性关节炎,他们出现在儿科皮肤科诊所,发现痤疮,银屑病样皮炎,和化脓性汗腺炎,其临床,遗传,实验室检查结果与PAMI综合征最为一致。我们进行了文献综述,以更好地描述这种罕见的皮肤病。识别在PAMI综合征中看到的独特皮肤发现可以帮助将其与其他炎症性疾病区分开来。加快诊断和治疗。
    PSTPIP1-associated myeloid-related proteinemia inflammatory (PAMI) syndrome is a rare autoinflammatory disorder often arising in pediatric patients. We present a case of an 18-year-old female with a past medical history of growth failure, immunoglobulin A nephropathy, and inflammatory arthritis who presented to a pediatric dermatology clinic with findings of acne, psoriasiform dermatitis, and hidradenitis suppurativa, whose clinical, genetic, and laboratory findings were most consistent with PAMI syndrome. We conducted a literature review to better characterize this rare condition in the context of dermatologic findings. Recognition of the distinctive skin findings seen in PAMI syndrome can help distinguish it from other inflammatory disorders, enabling expedited diagnosis and treatment.
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  • 文章类型: Case Reports
    COVID-19是一场始于2020年的全球大流行。大流行期间,患者出现COVID-19感染的各种皮肤表现。目前,COVID-19感染在全球范围内仍然存在,虽然程度较小。该病例报告描述了一名21岁的男性患者,他的手和脚踝外侧出现丘疹囊泡三天。该患者还出现了8天的呼吸道症状,并检测出COVID-19阳性。重要的是要了解与COVID-19相关的皮肤表现,因为这可以帮助诊断和理解病情。
    COVID-19 is a global pandemic that started in 2020. During the pandemic, patients have presented with various cutaneous manifestations of COVID-19 infections. Currently, COVID-19 infections are still present worldwide, although to a lesser extent. This case report describes a 21-year-old male patient who presented with papulovesicles on his hands and lateral aspects of his ankles for three days. The patient also experienced respiratory symptoms for eight days and tested positive for COVID-19. It is important to have an understanding of the skin manifestations associated with COVID-19, as this can aid in the diagnosis and comprehension of the condition.
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  • 文章类型: Case Reports
    Kindler综合征,一种罕见的遗传性大疱性表皮松解症分支,是一种常染色体隐性遗传疾病,其特征是婴儿期出现疼痛性水泡和出血性水泡。随着年龄的增长,水泡的爆发被看到下降,留下纤维化,伤痕累累,和纸一样的皮肤,和变性人的特征。到现在为止,全世界仅报告了大约400例这种疾病。本报告旨在利用发展中国家有限的资源讨论金德勒综合症的存在和诊断。它描述了巴基斯坦血统的年轻男性中临床诊断的Kindler综合征的存在,该综合征始于婴儿期,多年来具有各种临床特征。尽管基因分析仍然是金德勒综合征诊断的黄金标准,对于第三世界国家来说,依靠诊断临床标准仍然有助于建立Kindler综合征的诊断以进行进一步治疗,从我们的病人身上看到的.
    Kindler syndrome, a rare branching of inherited epidermolysis bullosa, is an autosomal recessive condition characterized by the eruption of painful blisters and hemorrhagic vesicles in infancy. With age, the eruption of blisters are seen to decline leaving behind fibrosed, scarred, and paper-like skin, and poikilodermic features. To this date, about 400 cases have been reported worldwide for this disease only. This report aims to discuss the presence and diagnosis of Kindler Syndrome using limited resources in developing countries. It describes the presence of clinically diagnosed Kindler Syndrome in a young male of Pakistani descent that started in infancy and presented with a variety of clinical features over the years. Even though genetic analysis remains the gold standard diagnostic for Kindler syndrome, for third world countries, relying on Diagnostic clinical criteria remains helpful in establishing a diagnosis of Kindler syndrome for further management, as seen in our patient.
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  • 文章类型: Case Reports
    干燥综合征(SS)以其口干和干眼症而闻名。岛外疾病的表现可能是变形蛋白,对临床医生构成挑战。特别是当典型的已知表现是不存在的。SS的皮肤受累是可变的,皮肤体征和症状可能是这种综合征的最初表现。液泡界面皮炎与皮肌炎和系统性红斑狼疮有关,但很少去SS。在这里,我们介绍了一例87岁的男性患者,他出现了广泛的瘙痒性红斑鳞片斑块,这些斑块对局部用皮质类固醇治疗无效,并停用了可能的不良药物.在抗SSA强阳性的情况下,活检显示空泡界面皮炎。羟氯喹治疗可有效解决斑块。
    Sjögren\'s syndrome (SS) has been widely known for its dry mouth and dry eyes presentation. Extraglandular disease manifestations may be protean and pose a challenge for clinicians, especially when the typical known manifestations are absent. Skin involvement of SS is variable, and cutaneous signs and symptoms may be the initial presentation of this syndrome. Vacuolar interface dermatitis has been linked to dermatomyositis and systemic lupus erythematosus, but rarely to SS. Herein, we present the case of an 87-year-old man who presented for widespread itchy erythematous scaly plaques that were refractory to topical corticosteroids as well as discontinuation of possible offending medications. A biopsy demonstrated vacuolar interface dermatitis in the setting of strongly positive anti-SSA. Hydroxychloroquine treatment was effective in resolving the plaques.
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  • 文章类型: Journal Article
    背景:皮肤肠外表现(EIM)发生在多达20%的IBD患者中。关于Sweet综合征(SS)作为IBD中罕见的皮肤EIM的临床过程的信息仅限于病例报告。我们提出了关于IBD中SS的发生和管理的最大回顾性队列研究。
    方法:对1980年以来的电子病历和纸质图表进行回顾性分析,在一个大型的四级医疗中心,以确定所有经组织病理学证实为SS的成年IBD患者。评估患者特征和临床结果。
    结果:确定了25名患有SS的IBD患者;评估了3名患有AZA诱导的SS的患者。大多数SS患者是女性。诊断时的中位年龄为47岁(IQR33-54岁),SS出现在IBD诊断后的中位年龄为6.4年。具有SS的IBD患者具有高的复杂性IBD表型(UC中75%的广泛结肠炎和CD中73%的狭窄或穿透性疾病,100%结肠受累),以及频繁的同时发生的EIM(60%)。SS与全球IBD疾病活动相关。糖皮质激素是治疗IBD的有效方法。SS复发率为36%。
    结论:与以前的病例报告相反,SS是我们队列中诊断为IBD后晚期发生的皮肤EIM,与全球IBD疾病活动平行。尽管AZA诱导的和IBD相关的SS都有效地治疗了皮质类固醇,区分它们与未来的IBD治疗策略有关。
    Cutaneous extra-intestinal manifestations (EIM) occur in up to 20% of patients with IBD. Information about Sweet syndrome (SS)\'s clinical course as a rare cutaneous EIM in IBD is limited to case reports. We present the largest retrospective cohort on the occurrence and management of SS in IBD.
    Electronic medical records and paper charts since 1980 were retrospectively reviewed at a large quaternary medical center to identify all adult IBD patients with histopathology-proven SS. Patient characteristics and clinical outcomes were evaluated.
    25 IBD patients with SS were identified; 3 patients were assessed to have AZA-induced SS. The majority of SS patients were female. Median age at diagnosis was 47 years (IQR 33-54 years) and SS appeared at a median of 6.4 years after IBD diagnosis. IBD patients with SS had a high rate of complicated IBD phenotypes (75% extensive colitis in UC and 73% stricturing or penetrating disease in CD, with 100% colonic involvement), as well as frequent co-occurring EIMs (60%). SS correlated with global IBD disease activity. Corticosteroids were an effective therapy for SS in IBD. Recurrence rate of SS was 36%.
    Contrary to previous case reports, SS was a cutaneous EIM occurring late after diagnosis of IBD in our cohort, with occurrences paralleling global IBD disease activity. Although AZA-induced and IBD-associated SS were both effectively treated with corticosteroids, distinguishing them is relevant for future IBD treatment strategies.
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  • 文章类型: Journal Article
    全膝关节置换术(TKA)后原发性皮肤弥漫性大B细胞淋巴瘤(PCDLBCL)很少见。
    分析PCDLBCL的皮肤表现并评估治疗效果和结果的文献很少。
    我们描述了TKA后PCDLBCL的一例,其皮肤肿块在手术部位周围发展,模仿假肢关节感染。此外,我们对29例报告的PCDLBCL病例进行了系统评价.该综述的主要终点是PCDLBCL的主要皮肤表现。次要终点包括PCDLBCL的治疗选择和最佳治疗方法。
    我们发现主要的皮肤表现包括浸润性皮肤病变,如斑疹,丘疹或结节,其中一些表现为溃疡或形成囊泡,皮下结节或两者兼有。治疗方案包括切除,放射治疗,化疗,在某些情况下,甚至注意到“警惕等待”作为自发回归。全身化疗是最常见的初始治疗方法,其中利妥昔单抗通常与环磷酰胺联合使用,阿霉素,长春新碱,和泼尼松(CHOP)化疗,接受全身性利妥昔单抗的患者的总生存期(OS)时间往往优于未接受全身性利妥昔单抗的患者.
    PCDLBCL是TKA后的一种罕见疾病,然而,仍然需要早期识别和区分感染。PCDLBCL患者可能受益于以利妥昔单抗为基础的化疗,提高存活率,尽管在某些情况下复发率高,OS时间有限。
    UNASSIGNED: Primary cutaneous diffuse large B-cell lymphoma (PCDLBCL) after total knee arthroplasty (TKA) is rare.
    UNASSIGNED: The literature that analyses the cutaneous manifestations of PCDLBCL and assesses the effect and the outcome of treatment is scarce.
    UNASSIGNED: We described a case of PCDLBCL after TKA, whose cutaneous mass develops around surgical sites, mimicking a prosthetic joint infection. In addition, we conducted a systematic review of 29 reported cases with PCDLBCL. Primary endpoint for the review was main cutaneous manifestations of PCDLBCL. Secondary endpoint included treatment options of PCDLBCL and optimal therapeutic method.
    UNASSIGNED: We found that the main cutaneous manifestations include infiltrative cutaneous lesions such as macules, papules or nodules, some of them presented as ulcerations or formation of vesicles, subcutaneous nodules or both. The treatment options include excision, radiotherapy, chemotherapy, and even \"watchful waiting\" as spontaneous regression was noted in some cases. Systemic chemotherapy is the most frequent initial treatment approach chosen, of which rituximab is often combined with cyclophosphamide, doxorubicin, vincristine, and prednisone (CHOP) chemotherapy and patients who received systemic rituximab tend to have a better overall survival (OS) time than those who did not.
    UNASSIGNED: PCDLBCL is a rare disease after TKA, however, an early recognition and distinguishing from infection is still needed. Patients with PCDLBCL may profit from rituximab-based chemotherapy, increasing the survival rate, despite the high relapse rate and limited OS time in some cases.
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  • 文章类型: Case Reports
    COVID-19的常见临床特征越来越为人所知,但医护人员很少描述皮肤表现。因此,需要审查COVID-19的皮肤表现,特别是在医护人员(HCW)中。本研究的目的是通过6例病例报告和文献综述,对HCW中COVID-19的皮肤表现进行全面综述。我们对病例报告进行了文献检索,使用PubMed和Google搜索引擎的原创和评论文章。我们纳入了提及COVID-19感染期间皮肤症状的书面英语研究。除了审查,从2020年9月至2021年1月,在突尼斯CharlesNicolle医院工作的HCW中收集了6例病例。在我们的审查中,除了我们的六个案例之外,我们还包括了17篇文章。论文类型分别为8项和9项研究中的病例系列和病例报告。在两项研究中报告了皮肤表现,包括两项HCW,除了我们的六个HCW系列。皮肤表现为冻疮样病变(44.5%),泛发性黄斑或斑丘疹(32.1%),丘疹泡疹和水痘样囊泡(11.5%),疼痛的肢端红紫色丘疹(4.6%),荨麻疹(2.8%),紫癜性或瘀点病变(2.3%),和网状利维多病变(0.45%)。在218个案例中,203例报告了病变的位置。绝大多数病变是在躯干上发现的,手,和脚。51例患者(23.4%)在躯干上出现病变。皮肤病变的发生时间是可变的。在诊断或出现COVID-19症状时,8.3%(18/218)的患者出现皮肤病变。在出现呼吸道症状或COVID-19诊断后,36.7%(80/218)的患者出现皮肤病变。在197例报告愈合时间的病例中,治愈时间长达24天。COVID-19与不同的皮肤表现有关,可能有不同的病理生理学和严重程度,一些在COVID-19症状之前,另一些在活动性疾病期间或病程后期发生。因此,提高对COVID-19皮肤表现的认识和及时识别对于使用COVID-19治疗HCW的职业医师很重要。
    Common clinical features of COVID-19 are increasingly known but cutaneous manifestations are rarely described among healthcare workers. A review of cutaneous manifestations of COVID-19 especially among healthcare workers (HCW) is thus required. The aim of our current study was to provide a comprehensive review of cutaneous manifestations of COVID-19 among HCW through six case reports and a literature review. We conducted a literature search for cases reports, original and review articles using PubMed and the Google search engines. We included the written English studies that mentioned cutaneous symptoms during COVID-19 infection. In addition to the review, 6 cases were collected among the HCW working at the Charles Nicolle Hospital of Tunis from September 2020 to January 2021. In our review, we included 17 articles in addition to our six cases. The type of papers was case series and case reports in respectively 8 and 9 studies. Skin manifestations were reported in two studies including two HCW, in addition to our series of six HCW. Skin manifestations were chilblain-like lesions (44.5%), a generalized macular or maculopapular exanthem (32.1%), a papulovesicular rash and chicken pox-like vesicles (11.5%), painful acral red-purple papules (4.6%), urticaria (2.8%), purpuric or petechial lesions (2.3%), and livedo reticularis lesions (0.45%). Of the 218 cases, 203 cases have reported the location of lesions. The very great majority of lesions were found on the trunk, hands, and feet. Fifty-one patients (23.4%) experienced lesions on the trunk. The time of onset of cutaneous lesions was variable. At the diagnosis or the onset of COVID-19 symptoms, 8.3% (18/218) of patients presented with cutaneous lesions. After the onset of respiratory symptoms or COVID-19 diagnosis, 36.7% (80/218) of patients presented with cutaneous lesions. Of the 197 cases with reported healing times, healing times were up to 24 days. COVID-19 has been associated with different cutaneous manifestations, likely of varying pathophysiology and severity, some preceding COVID-19 symptomatology and others occurring during active disease or later in the course. Thus, heightened awareness and timely recognition of cutaneous manifestations in COVID-19 are important for occupational physicians treating HCW with COVID-19.
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  • 文章类型: Case Reports
    在SARS-CoV-2大流行期间,据报道,与COVID-19感染相关的各种皮疹,包括荨麻疹.荨麻疹是一种有限的,通常是良性的疾病,表现为瘙痒性风团,有或没有水肿。一名39岁的妇女在两天内出现了手臂上瘙痒的皮疹,蔓延到躯干和面部,接着是头痛,恶心,呕吐,腹部不适,腹泻,肌痛,关节痛,嗅觉缺失,和消化不良三天。发烧,干咳,在咨询当天就开始了吞咽痛.患者有高血压病史,但否认有特应性病史,类似以前的介绍,或最近摄入新药物。SARS-CoV-2COVID-19PCR检测呈阳性。她服用口服抗组胺药治疗瘙痒并出院。在两周后的随访中,患者无症状,在症状出现的第7天皮疹完全缓解。了解COVID-19的皮肤表现可以帮助早期识别这种疾病并防止误诊。COVID-19的皮肤表现被认为与疾病的严重程度有关,但需要数据来研究COVID-19的皮肤病学表现的任何预后价值.
    During the SARS-CoV-2 pandemic, various rashes associated with COVID-19 infection have been reported, including urticaria. Urticaria is a limited and usually benign condition, presenting as pruritic wheals, with or without edema. A 39-year-old woman presented with a pruritic rash on her arms spreading to her trunk and face over two days, followed by headache, nausea, vomiting, abdominal discomfort, diarrhea, myalgia, arthralgia, anosmia, and dyspepsia for three days. Fever, dry cough, and odynophagia started on the day of the consult. The patient had a history of hypertension but denied a history of atopic conditions, similar previous presentations, or recent ingestion of new medications. SARS-CoV-2 COVID-19 PCR testing was positive. She was prescribed oral antihistamine for the itching and was discharged. During a follow-up after two weeks, the patient was asymptomatic with complete resolution of the rash on day 7 of symptoms. Knowing the cutaneous manifestations of COVID-19 can aid in the early identification of this disease and prevent misdiagnosis. The presence of cutaneous manifestations in COVID-19 is suggested to be related to disease severity, but data are needed to study any prognostic value of dermatologic manifestations in COVID-19.
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  • 文章类型: Journal Article
    冠状病毒病的主要临床表现是呼吸道感染,从无症状的携带者状态到严重的间质性肺炎。最常见的症状是发烧,干咳,疲劳,恶心,腹泻,失去气味或味道。最近的报道表明可能出现几种非特异性皮肤病学症状。这些包括荨麻疹,斑丘疹病变,涉及瘀斑的血管病变,紫癜和网状livedo。皮肤病变的发作可能先于全面发展的COVID-19,或者仍然是该疾病的唯一表现(特别是在年轻患者中)。关注皮肤病学症状对于防止SARS-CoV-2的传播可能至关重要。然而,明确评估COVID-19的皮肤表现需要皮肤科医生进行更多的研究和案例研究。
    Primary clinical manifestations of coronavirus disease are respiratory tract infections, ranging from an asymptomatic carrier state to severe interstitial pneumonia. The most common symptoms are fever, dry cough, fatigue, nausea, diarrhea, and loss of smell or taste. Recent reports indicate the possibility of several nonspecific dermatological symptoms. These include urticaria, maculopapular lesions, vascular lesions involving petechiae, purpura and livedo reticularis. The onset of cutaneous lesions may precede full-blown COVID-19 or remain the only manifestation of the disease (especially in young patients). Focusing on dermatological symptoms may be crucial for preventing the spread of SARS-CoV-2. However, unambiguous assessment of cutaneous manifestations of COVID-19 requires more research and case studies conducted by dermatologists.
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  • 文章类型: Case Reports
    未经评估:SARSCOV2感染是一种具有挑战性的流行病,已经影响了数百万死亡率很高的人。除了典型的呼吸道症状,它也会导致可变的皮肤损伤,如血管性紫癜在一些特殊情况下。
    方法:我们报告了一例因SARSCOV2感染而入院的60岁女性,症状开始后D20时出现血管性紫癜,标志着这种演变.
    结论:与SARSCOV2感染相关的皮肤表现是多态的。血管性紫癜就是其中之一。它的诊断是根据各种论点而保留的,这使得医生诊断它是一个真正的挑战。该疾病的管理基于对症治疗。临床演变是,总的来说,有利的。
    结论:虽然罕见且仍未完全解释,已经描述了SARSCOV2感染期间的皮肤受累。它不应该被忽视,应该早期诊断和适当治疗,尤其是无症状患者。
    UNASSIGNED: The SARS COV2 infection is a challenging pandemic that has affected millions of people with a very high mortality rate. In addition to the typical respiratory symptoms, it can also cause variable skin lesions, such as vascular purpura in some exceptional cases.
    METHODS: We report the case of a 60-year-old woman who was admitted for a SARS COV2 infection, the evolution was marked by the appearance of a vascular purpura at D20 after the beginning of the symptoms.
    CONCLUSIONS: The cutaneous manifestations associated with the SARS COV2 infection are polymorphic. Vascular purpura is one of them. Its diagnosis is retained in the light of a combination of arguments, which makes it a real challenge for the physician to diagnose it. The management of the disease is based on a symptomatic treatment. The clinical evolution is, in general, favorable.
    CONCLUSIONS: Although rare and still not fully explained, skin involvement during SARS COV2 infection has been described. It should not be neglected and it should be diagnosed early and treated appropriately, especially in asymptomatic patients.
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