peau d’orange

  • 文章类型: Journal Article
    目的:血管样条纹(AS)是一种罕见的视网膜疾病,当并发脉络膜新生血管(CNV)时,视力受损。它们代表布鲁赫膜水平的裂纹状开裂。这篇客观的叙事综述旨在提供病理生理学的概述,目前的治疗方式,以及在这种情况下的未来前景。材料与方法:使用“PubMed”进行文献检索,\"WebofScience\",\"Scopus\",“科学直接”,“谷歌学者”,\"medRxiv\",和“bioRxiv。“结果:AS可能是特发性的,但它们也与系统条件有关,比如弹性假性黄瘤,遗传性血红蛋白病,或者Paget的病.目前,主要治疗方法是使用抗血管内皮生长因子(anti-VEGF)治疗继发性CNV,这是在这种情况下观察到的主要并发症。如果CNV被及时检测和治疗,ASs患者有很好的机会维持功能性视力。其他治疗方式已经尝试,但显示出有限的益处,因此,没有被更广泛地接受。结论:总之,虽然目前还没有明确的治疗方法,抗VEGF治疗继发性CNV为维持AS患者的功能性视力提供了机会,前提是早期检测和治疗CNV。
    Aim: Angioid streaks (ASs) are a rare retinal condition and compromise visual acuity when complicated with choroidal neovascularization (CNV). They represent crack-like dehiscences at the level of the Bruch\'s membrane. This objective narrative review aims to provide an overview of pathophysiology, current treatment modalities, and future perspectives on this condition. Materials and Methods: A literature search was performed using \"PubMed\", \"Web of Science\", \"Scopus\", \"ScienceDirect\", \"Google Scholar\", \"medRxiv\", and \"bioRxiv.\" Results: ASs may be idiopathic, but they are also associated with systemic conditions, such as pseudoxanthoma elasticum, hereditary hemoglobinopathies, or Paget\'s disease. Currently, the main treatment is the use of anti-vascular endothelial growth factors (anti-VEGF) to treat secondary CNV, which is the major complication observed in this condition. If CNV is detected and treated promptly, patients with ASs have a good chance of maintaining functional vision. Other treatment modalities have been tried but have shown limited benefit and, therefore, have not managed to be more widely accepted. Conclusion: In summary, although there is no definitive cure yet, the use of anti-VEGF treatment for secondary CNV has provided the opportunity to maintain functional vision in individuals with AS, provided that CNV is detected and treated early.
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  • 文章类型: Journal Article
    描述弹性假性黄瘤(PXE)患者布鲁赫膜(BM)钙化的自然史。
    回顾性队列研究。
    120名年龄小于50岁的PXE患者的双眼,其中78人在超过1年后进行了随访成像。
    所有患者都接受了多模态成像,包括彩色眼底摄影,近红外反射(NIR)成像,和后期吲哚菁绿血管造影(ICGA)。我们在NIR上确定了从视盘到橙色中心和时间边界的距离,以水平视神经盘直径(ODD)表示。最长血管样条纹的长度分为5个区域。
    橙色的特定年龄变化,血管样条纹,和ICGA荧光不足作为BM钙化程度的替代标志物。
    在横截面分析中,血管样条纹较长与年龄增长相关(趋势P<0.001).橙色的时间边界显示出与年龄增长的弱关联(β=0.02;95%置信区间[CI],0.00-0.04),而中央边界显示出强关联(β=0.12;95%CI,0.09-0.15)。纵向分析显示,中心边界向外围的中值位移为每年0.08ODD(四分位间距[IQR],0.00-0.17;P<0.001)。这种转变在20岁以下的患者中更为明显(每年0.12ODD[IQR,0.08-0.28])比40岁以上的患者(每年0.07ODD[IQR,-0.05至0.15])。时间边界在随访期间没有移位(P=0.69)。在156只眼中的39只(25%)中检测到新的或正在生长的血管样条纹。ICGA上的低荧光区域仅在第四个或第五个十年中可见,并且与更长的血管样条纹相关。
    在PXE患者中,斑点的BM钙化在一生中缓慢融合。橙色的时间边界的位置保持相当恒定,而中央边界转移到外围。这表明存在用于BM钙化的预定区域。较大的ICGA低荧光区域与年龄较大和较长的血管样条纹相关,这意味着它取决于BM钙化的程度。
    UNASSIGNED: To describe the natural history of Bruch\'s membrane (BM) calcification in patients with pseudoxanthoma elasticum (PXE).
    UNASSIGNED: Retrospective cohort study.
    UNASSIGNED: Both eyes of 120 PXE patients younger than 50 years, 78 of whom had follow-up imaging after more than 1 year.
    UNASSIGNED: All patients underwent multimodal imaging, including color fundus photography, near-infrared reflectance (NIR) imaging, and late phase indocyanine green angiography (ICGA). We determined the distance from the optic disc to the central and temporal border of peau d\'orange on NIR, expressed in horizontal optic disc diameter (ODD). The length of the longest angioid streak was classified into 5 zones.
    UNASSIGNED: Age-specific changes of peau d\'orange, angioid streaks, and ICGA hypofluorescence as surrogate markers for the extent of BM calcification.
    UNASSIGNED: In cross-sectional analysis, longer angioid streaks were associated with increasing age (P < 0.001 for trend). The temporal border of peau d\'orange showed a weak association with increasing age (β = 0.02; 95% confidence interval [CI], 0.00-0.04), whereas the central border showed a strong association (β = 0.12; 95% CI, 0.09-0.15). Longitudinal analysis revealed a median shift of the central border to the periphery of 0.08 ODD per year (interquartile range [IQR], 0.00-0.17; P < 0.001). This shift was more pronounced in patients younger than 20 years (0.12 ODD per year [IQR, 0.08-0.28]) than in patients older than 40 years (0.07 ODD per year [IQR, -0.05 to 0.15]). The temporal border did not shift during follow-up (P = 0.69). New or growing angioid streaks were detected in 39 of 156 eyes (25%). The hypofluorescent area on ICGA was visible only in the fourth or fifth decade and correlated with longer angioid streaks.
    UNASSIGNED: In PXE patients, the speckled BM calcification slowly confluences during life. The location of the temporal border of peau d\'orange remains rather constant, whereas the central border shifts to the periphery. This suggests the presence of a predetermined area for BM calcification. A larger ICGA hypofluorescent area correlates with older age and longer angioid streaks, which implies that it depends on the degree of BM calcification.
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  • 文章类型: Journal Article
    目的:这篇叙述性综述的目的是总结炎性乳腺癌(IBC)的误诊或延迟诊断的原因以及快速诊断的策略。
    背景:IBC患者经常报告该病最初被误诊,最常见的是乳腺炎。
    方法:我们通过使用序贯PubMed搜索标准(包括IBC乳腺症状)对这一具有挑战性的诊断进行了综述,IBC诊断,和IBC成像模式,以增加作者对IBC的认识。从PubMed搜索中确定的手稿和手稿审阅者中添加了其他参考文献。
    结论:多种因素导致IBC的延迟诊断。一个重要因素是IBC并不常见,而许多通才在乳腺皮肤症状的鉴别诊断中可能并没有意识到这一点。IBC的一些特征导致乳房X线照相术对其检测的敏感性低,因此诊断是基于临床因素,因此是主观的。演示文稿可以是多种多样的;经典的教科书图像不能捕捉到整个肤色中出现的体征和症状的范围,可能会导致非典型演示文稿患者的漏诊。事实上,美国癌症联合委员会的分期系统,这需要乳房皮肤红斑来诊断,可能排除有明显整体乳房皮肤发现但未明确红色的患者。我们提出了一种适应的算法,用于处理未诊断的炎性乳房,以确保及时准确地诊断IBC。我们断言Frank,如果这些体征的时间和活检发现一致,则在弥漫性乳腺恶性肿瘤的增大的乳房中出现的非红斑性整体皮肤体征足以诊断IBC.我们还提供了通过全球乳房皮肤体征识别的非典型IBC的图像,包括橙色,在没有明显红斑的情况下与IBC一致。
    OBJECTIVE: The purpose of this narrative review is to summarize the contributors to misdiagnosis or delayed diagnosis of inflammatory breast cancer (IBC) and strategies for expedient diagnosis.
    BACKGROUND: Patients with IBC often report the disease as initially being misdiagnosed, most commonly as mastitis.
    METHODS: We reviewed the literature on this challenging diagnosis by using sequential PubMed search criteria including IBC breast symptoms, IBC diagnosis, and IBC imaging modalities to augment the authors\' knowledge of IBC. Other references were added from the manuscripts identified in the PubMed searches and from manuscript reviewers.
    CONCLUSIONS: Several factors contribute to the delayed diagnosis of IBC. One important factor is that IBC is uncommon, and many generalists may not be aware of it in the differential diagnosis of breast skin symptoms. Several features of IBC contribute to the low sensitivity of mammography for its detection, and so the diagnosis is based on clinical factors and is thereby subjective. The presentation can be highly varied; classic textbook images that do not capture the range of presenting signs and symptoms across skin tones may contribute to missed diagnoses in patients with atypical presentations. In fact, the staging system of the American Joint Committee on Cancer, which requires erythema of the breast skin for diagnosis, may exclude patients with obvious global breast skin findings that are not explicitly red. We present an adapted algorithm for working up the undiagnosed inflammatory breast to ensure the timely and accurate diagnosis of IBC. We assert that frank, non-erythematous global skin signs in an enlarged breast with diffuse breast malignancy are sufficient to diagnose IBC if the timing of these signs and findings on biopsy are consistent. We further provide images of atypical IBC identified by global breast skin signs, including peau d\'orange, consistent with IBC in the absence of frank erythema.
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  • 文章类型: Journal Article
    Purpose: To evaluate the fundus phenotype of young patients affected with Pseudoxantoma Elasticum (PXE). Materials and Methods: Retrospective case series of five young PXE patients. Clinical data, ultra-widefield imaging (color, red-free (RF), choroidal (Ch) and fundus autofluorescence (FAF)) and OCT examination were collected. Diagnosis was confirmed by the characteristic histopathological abnormalities in skin biopsies and genetic testing results. Results: Five patients, 2 males and 3 females (mean age 16 years, range 12-20 years) were included in our study. The visual acuity was 20/20 in all subjects. Fundus evaluation revealed peau d\'orange in all patients: multiple, yellowish/white round lesions, scattered from the posterior pole to the mid-peripheral retina of each eye. Ultra-wide field imaging allows us to capture and describe the entire area of coquille d\'oeuf/peau d\'orange in a single picture, facilitating their identification and discrimination. Angiod streaks were visible in both eyes of four patients. In one patient optic disc drusen were detected in both eyes. All patients presented comet lesions. Conclusions: PXE-related retinopathy findings: peau d\'orange/coquille d\'oeuf, angioid streaks, comet lesions and drusen of the optic disc were present early in PXE patients. The early detection of coquille d\'oeuf/peau d\'orange revealed a preferable area into midperiphery where Bruch\'s membrane will be more likely to be affected.
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  • 文章类型: Journal Article
    BACKGROUND: Pseudoxanthoma elasticum (PXE) is a rare autosomal recessive disorder caused by mutations in the ABCC6 gene, resulting in various retinal lesions, among other systemic manifestations. Visual loss may occur in PXE, most commonly caused by choroidal neovascularization and macular atrophy, but little is known about the consequences of fundus pulverulentus (FP) in PXE. The aim of this study was to evaluate ophthalmic outcomes in patients with FP associated with PXE in a large series of PXE patients.
    METHODS: In a retrospective observational study, ophthalmic outcomes were compared between two groups of age-matched patients with genetically and pathologically confirmed PXE: one group with FP versus one without FP. All included patients underwent thorough clinical examination. Further investigation (optical coherence tomography (OCT), Cirrhus, Zeiss Germany, and/or fluorescein/indocyanin green angiography) was performed in cases of suspected choroidal neovascularization (CNV).
    RESULTS: The study included 13 PXE patients with FP (group 1: 8 men and 5 women, aged 45-65 years) and 47 age-matched PXE patients without FP (group 2: 19 men and 28 women). Mean patient follow-up was 63 months (range 0-132 months). Subretinal fibrosis (SRF) was more frequently associated with FP (9/26 eyes, 34.6%), compared to absence of FP (4/94, 4.2%) (p = 0.0001). Independently of SRF, FP can evolve into deep macular atrophy and/or CNV with dramatic consequences for central vision.
    CONCLUSIONS: Fundus pulverulentus may occur in PXE and is most commonly associated with subretinal fibrosis in the posterior pole and visual loss by macular atrophy even in the absence of CNV.
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  • 文章类型: Journal Article
    We reviewed 200 patient records from 2009 to 2015 of individuals who presented at the authors offices for facial wrinkle reduction and who on physical examination had rhytides, peau d\'orange, or thin dermis and underwent novel adipose slurry injections. The patients were evaluated at week 2, 1, 3 and 6 months revealing an 80, 60 and 40% improvement in rhytides and dermis density, respectively. We present the protocol and description of a reasonable alternative or adjunct to manufactured fillers that is comprised of autologous adipose, stem cells, and growth factor slurry. The slurry is easily produced and injected at the bedside in the office setting, and has remarkable and reproducible positive outcomes in skin tone, color, texture, and rhytides as judged by both patient and physician.
    METHODS: This journal requires that authors assign a level of evidence to each article. For a full description of these Evidence-Based Medicine ratings, please refer to the Table of Contents or the online Instructions to Authors www.springer.com/00266 .
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