macular amyloidosis

  • 文章类型: Journal Article
    黄斑淀粉样变性(MA)中皮肤的不吸引人的外观干扰了患者的生活质量,治疗这种疾病一直具有挑战性。
    为了比较15%三氯乙酸(TCA)果皮的功效和安全性,35%乙醇酸(GA)果皮,和Q开关Nd-YAG激光器在MA。
    打开,前瞻性研究进行了2年,在满足标准之后,随机分为三组.A组:15%TCA剥离;B组:35%GA剥离;C组:Q开关Nd-YAG激光。每月一次重复该程序,共进行六次。每次访问,患者根据以下参数进行评分:颜色评分,视觉模拟评分(VAS),医师全球评估(PGA)量表,主观改进,和不利影响。
    共有75名患者(每组25名患者)完成了治疗。研究人群的平均年龄为35.68±9.8岁,男女比例为11.5:1。A组颜色评分的平均变化(1.68)更大,其次是C组(1.4),其次是B组(1.16)。类似的结果是通过主观改进注意到的,VAS,和PGA。A组的不良事件更多,其次是C组,其次是B组。无患者出现永久性不良事件。
    TCA剥离显示优于Nd-YAG激光和乙醇酸剥离的结果。TCA剥离具有良好的可接受性,具有成本效益,应被视为一种治疗方式。
    UNASSIGNED: The unappealing appearance of skin in macular amyloidosis (MA) interferes with the patient\'s quality of life, and treating the condition has been challenging.
    UNASSIGNED: To compare the efficacy and safety of 15% trichloroacetic acid (TCA) peel, 35% glycolic acid (GA) peel, and Q-switched Nd-YAG Laser in MA.
    UNASSIGNED: Open, prospective study was conducted over 2 years, where after fulfilling the criteria, randomization was done into three groups. Group A: 15% TCA peel; Group B: 35% GA peel; Group C: Q-switched Nd-YAG Laser. Procedures were repeated at monthly intervals for six sittings. On each visit, patients were scored on the following parameters: color score, visual analog score (VAS), physician global assessment (PGA) scale, subjective improvement, and adverse effects.
    UNASSIGNED: A total of 75 patients (25 patients in each group) completed the treatment. The mean age of study-population was 35.68 ± 9.8 years, with female to male ratio of 11.5:1. The mean change in color score (1.68) was more by Group A, followed by Group C (1.4), followed by Group B (1.16). Similar results were noted by subjective improvement, VAS, and PGA. Adverse events were more in Group A, followed by Group C, followed by Group B. No patient showed permanent adverse events.
    UNASSIGNED: TCA-peel showed superior results over Nd-YAG laser and glycolic-acid peel. TCA-peel being cost-effective with excellent acceptability should be considered a treatment modality.
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  • 文章类型: Journal Article
    淀粉样蛋白沉积可能是许多慢性疾病的原因。原发性局部皮肤淀粉样变性(PLCA)是一种慢性皮肤病,在乳头状真皮中存在淀粉样蛋白沉积。最常见的角质形成细胞衍生形式的PLCA包括黄斑(MA),地衣(LA),和双相(BA)淀粉样变性。亚洲人口中PLCA的估计患病率为0.98/10,000,高于欧洲人口;因此,白种人人群中PLCA的流行病学数据有限.我们进行了一项回顾性单中心研究,分析了中欧PLCA人群的流行病学特征。关于年龄的流行病学数据,性别,皮肤光型(Fitzpatrick量表I-VI),疾病持续时间,合并症,过敏症的历史,收集PLCA家族史。临床特征,PLCA病变的定位,还分析了应用的疗法和治疗结果.还评估了皮肤特征。共纳入41例诊断为PLCA的患者,22岁出现黄斑,18与地衣,1为双相淀粉样变性。男女比例为16/25,诊断时的平均年龄为54.6±15.2岁(范围27-87岁)。在MA中,PLCA发作的平均年龄为53±16.1岁(范围19-79岁),洛杉矶46.7±18.2年(范围14-73年),在BA工作了26年。MA的肩胛骨间区域和LA的下肢伸肌表面被证明是与定位相关的区域。在我们的中心,应用了广泛的治疗选择,在所有类型的PLCA中,处方最多的是局部用皮质类固醇。我们提出了一个回顾,中欧地区PLCA流行病学的单中心研究。通过检查大量PLCA患者的医疗数据,我们将我们的流行病学数据与亚洲PLCA人群的流行病学数据进行了比较.由于这种情况的罕见,需要进一步的随机对照试验和指南来改善治疗结果.
    Amyloid deposits can be the cause of many chronic diseases. Primary localized cutaneous amyloidosis (PLCA) is a chronic dermatologic condition with amyloid deposits in the papillary dermis. The most common types of the keratinocyte-derived form of PLCA include macular (MA), lichen (LA), and biphasic (BA) amyloidosis. The estimated prevalence of PLCA in the Asian population is 0.98/10,000, which is higher than in the European population; thus, epidemiologic data on PLCA in the Caucasian population are limited. We performed a retrospective single-center study analyzing epidemiologic characteristics of a Central European PLCA population. Epidemiologic data regarding age, sex, skin phototype (Fitzpatrick scale I-VI), disease duration, comorbidities, history of atopy, and family history of PLCA were collected. Clinical characteristics, localization of PLCA lesions, applied therapies and treatment outcomes were also analyzed. Dermoscopic characteristics were also evaluated. A total of 41 patients diagnosed with PLCA were included, with 22 presenting with macular, 18 with lichen, and 1 with biphasic amyloidosis. The male/female ratio was 16/25, and mean age at diagnosis was 54.6 ± 15.2 years (range 27-87 years). The mean age at the onset of PLCA was 53 ± 16.1 years (range 19-79 years) in MA, 46.7 ± 18.2 years (range 14-73 years) in LA, and 26 years in BA. The interscapular region in MA and the extensor surface of the lower extremities in LA proved to be localization-related areas. In our center, a wide range of therapeutic options was applied, with the most prescribed being topical corticosteroids in all types of PLCA. We presented a retrospective, monocentric study on the epidemiology of PLCA in the Central European region. By examining the medical data of a significant number of PLCA patients, we compared our epidemiologic data with that of the Asian PLCA population. Due to the rarity of the condition, further randomized controlled trials and guidelines are needed to improve therapeutic outcomes.
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  • 文章类型: Case Reports
    黄斑淀粉样变性是一种常见类型的原发性局限性皮肤淀粉样变性。我们提供了一例病例报告,其中一名74岁的患者没有明显的既往病史,对其进行了一年多的深色黄斑和瘙痒评估。在考试中,上背部和下背部的滤泡型棕色斑疹,双边肩,并注意到双侧背侧上臂。滤泡性黄斑的形态和分布异常,所以差异包括毛囊扁平苔藓,滤泡性湿疹,和黄斑淀粉样变性。穿刺活检显示嗜酸性原纤维材料沉积,并在乳头状真皮中伴有色素性失禁,与黄斑淀粉样变性一致。此外,在网状真皮中,附件结构被捕获,附件周围脂肪萎缩。在黄斑淀粉样变性角蛋白中,中间细丝如细胞角蛋白作为淀粉样蛋白前体,沉积在浅层真皮中。典型地,黄斑淀粉样变性表现为色素沉着的黄斑或斑块,通常呈“波纹”线性模式。由于非典型的滤泡受累,该病例突出了黄斑淀粉样变性的罕见表现,并强调了局部皮肤淀粉样变性的各种表现。此外,讨论了Janus激酶抑制剂等新的治疗选择及其在病理途径中的潜在作用。
    Macular amyloidosis is a common type of primary localized cutaneous amyloidosis. We present a case report of a 74-year-old patient with no significant past medical history who was evaluated for dark macules and pruritus for over a year. On exam, follicular-based brown macules on the upper and lower back, bilateral shoulders, and bilateral dorsal upper arms were noted. The morphology and distribution of follicular-based macules was unusual, so the differential included follicular lichen planus, follicular eczema, and macular amyloidosis. Punch biopsy showed deposits of eosinophilic fibrillary material along with pigmentary incontinence in the papillary dermis, consistent with macular amyloidosis. Additionally, there was some trapping of the adnexal structures with atrophy of the periadnexal fat in the reticular dermis. In macular amyloidosis keratin, intermediate filaments such as cytokeratin serve as the amyloid precursors which deposit in the superficial dermis. Characteristically, macular amyloidosis presents as hyperpigmented macules or patches, often in a \"rippled\" linear pattern. This case highlights a rare presentation of macular amyloidosis because of the atypical follicular involvement and emphasizes the variety of presentations for localized cutaneous amyloidosis. Additionally, new treatment options such as Janus Kinase inhibitors and their potential role in the pathological pathway are discussed.
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  • 文章类型: Case Reports
    Primary cutaneous amyloidosis (PCA) is characterized by the extracellular deposition of amyloid in the skin without systemic involvement. It comprises several clinical variants, the most common of which are macular amyloidosis (MA) and lichen amyloidosis (LA). PCA is frequently observed in Asians, while it is considered to be very rare in Caucasians. In the latter population, the condition often poses a diagnostic challenge. Dermoscopy has already been proved to be a useful, non-invasive diagnostic tool in various non-neoplastic skin diseases. In the paper, we present three Caucasian patients (skin phototypes I-II) with histologically confirmed LA. Under dermoscopy, central white hubs with grayish-brown dots and globules were observed in all three cases. Vascular structures were present in two cases and had the morphology of red globules and thick, unfocused branching lines intersecting the white hubs. A comprehensive review of the literature retrieved twelve papers presenting the dermoscopic features of PCA, including five articles on the dermoscopy of LA. The vast majority of these studies have been conducted on the Asian population, and there is a lack of data on the dermoscopic findings for patients with skin type I or II. The literature review revealed that MA and LA share several dermoscopic similarities (the presence of a white central hub and grayish dots), but also display distinct features. Compared to the dermoscopic features of LA in darker skin phototypes, our patients presented less pronounced pigmentation and more evident vascular structures. Nevertheless, further studies are needed in order to reliably evaluate the dermoscopic features of PCA in various ethnicities.
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  • 文章类型: Journal Article
    原发性局部皮肤淀粉样变性(PLCA)在皮肤中引起细胞外蛋白质沉积。临床上分为黄斑淀粉样变性,苔藓淀粉样变性和结节性淀粉样变性。PLCA的非典型介绍使诊断具有挑战性,需要活检以确认淀粉样蛋白沉积在上乳头状真皮中。
    这项研究使用FotoFinder皮肤镜检查来表征扁平苔藓和黄斑淀粉样变性,并将皮肤镜检查特征与组织病理学结果相关联。
    这项横断面研究纳入了临床和组织病理学诊断为PLCA的患者。使用FotoFinder皮肤镜进行皮肤镜检查,它提供了从20×到140×的放大范围。
    共30名患者纳入研究。MA的常见皮肤镜样为白色或棕色中央枢纽,洛杉矶的常见模式是白色无结构,疤痕状区域和中央枢纽。新的皮肤镜检查结果是MA中的百合外观和LA中的白色玫瑰花。
    皮肤镜检查在证明PLCA的特征性发现中起着关键作用。这些发现与组织病理学密切相关,从而避免不必要的活检以达到PLCA的准确诊断。
    UNASSIGNED: Primary localized cutaneous amyloidosis (PLCA) causes extracellular proteinaceous deposits in skin. It is clinically divided into macular amyloidosis, lichen amyloidosis and nodular amyloidosis. Atypical presentations of PLCA make the diagnosis challenging, requiring biopsy to confirm amyloid deposition in the upper papillary dermis.
    UNASSIGNED: This study used FotoFinder dermoscopy to characterize lichen and macular amyloidosis and correlated the dermoscopic features with histopathological findings.
    UNASSIGNED: This cross-sectional study enrolled patients with a clinical and histopathological diagnosis of PLCA. Dermoscopic examination was performed using the FotoFinder dermoscope, which provides a range of magnification from 20× to 140×.
    UNASSIGNED: A total of 30 patients were included in the study. Common dermoscopic patterns of MA were white or brown central hubs, and common patterns of LA were white structureless, scar-like areas and central hubs. New dermoscopic findings were a day lily appearance in MA and white rosettes in LA.
    UNASSIGNED: Dermoscopy plays a pivotal role in demonstrating characteristic findings of PLCA. These findings were well corelated with histopathology, thus avoiding unnecessary biopsy for arriving at an accurate diagnosis of PLCA.
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  • 文章类型: Journal Article
    The aim of the present study was to investigate the clinical association between rippled pattern pigmentation and the positivity of histopathological analysis for amyloid. A total of 50 patients (90% women) with rippled pattern pigmentation referring to Dermatology Clinic of Shahid Faghihi Hospital, Shiraz, Iran, in 2015 participated in this study. Two biopsies were performed for all the cases from the most frequently affected sites. The specimens were evaluated for amyloid deposits with hematoxylin-eosin (H&E), Congo red (CR), and crystal violet (CV) stains. The upper back was the most frequently affected area in the patients. Family history (28%), atopy (14%), pruritus as a common finding (86%), and history of friction (54%) were positive. The prevalence of disease was higher in patients with skin photo Type 3. Amyloid deposit was not detected in most patients by these stains. No statistically significant difference was found between the amyloid positive cases stained with H&E, CR, and CV (p-value > 0.05). Only the difference in positive results between biopsy number 2 and the total biopsy (1 and 2) was significant (p-value < .05). In conclusion, it seems that it is useful to increase the number of biopsies and other more sensitive staining methods to detect small focal amyloid deposits.
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  • 文章类型: Clinical Trial
    Macular amyloidosis (MA) is a common form of primary localized cutaneous amyloidosis, characterized by the eruption of brown pigments of the skin with a rippled pattern. MA can be of cosmetic concern for patients, and its treatment is challenging. In this study, we aimed to find new effective approaches for MA treatment. A total of 39 patients with the clinical diagnosis of MA were treated with two types of laser therapy, and the effectiveness of each approach was examined. Fractional Q-switched 10.64 nm neodymium-doped yttrium aluminum garnet (Nd:YAG) laser therapy was compared with a combination of fractional Q-switched 10.64 nm Nd:YAG laser and long-pulsed fractional erbium:YAG laser therapy. Melanin biometric measurements were performed using a Mexameter, objective image-based evaluation was carried out, and the itching score and patient satisfaction were examined. Mexameter-based analysis showed that both types of laser therapy were effective in the treatment of MA, causing a significant decrease in the amount of melanin in the treated areas (P < 0.05). Also, combination of two types of laser therapy was significantly more effective than one type alone (P < 0.05). The itching score significantly decreased in patients undergoing a combination of laser therapies. Also, a positive correlation was observed between the amount of melanin and degree of itching in the treated areas. Moreover, analysis of patient satisfaction showed that more than 90% of patients had excellent satisfaction with combination laser therapy. The results confirmed the significant positive effects of both fractional Nd:YAG laser alone and in combination with fractional erbium:YAG laser therapy considering the reduction in melanin content; however, combination of two types of laser therapy was more effective than one type alone. Trial registration: IRCT20080901001159N23.
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  • 文章类型: Comparative Study
    背景:没有局部和全身治疗被证明对皮肤黄斑淀粉样变性(CMA)有效。通过激光物理去除沉积的蛋白质可能是更好的选择。
    目的:比较包括脉冲染料激光(PDL)在内的不同激光的功效,1064模式的调QNd-YAG,CO2,以及用于治疗CMA的CO2和Q开关Nd:YAG激光器的组合。
    方法:本研究包括17例临床CMA和组织病理学证实的成年女性患者。用PDL同时处理四个接近1×1cm2的区域,调QNd-YAG,CO2和组合CO2和Nd-YAG激光器。用于585nm的PDL的能量为9J/cm2,光斑尺寸为5mm。用于1064nm模式的Nd-YAG激光器的能量为5焦耳/cm2,光斑尺寸为4mm,和10赫兹的频率。用于CO2激光的能量对于第一和第二通路是4和3.5微焦耳。
    结果:PDL治疗后没有获得理想的结果。Q开关Nd-YAG仅对3例患者有效。清除所有用CO2激光处理的区域。
    结论:CO2激光似乎是CMA最有效的激光治疗方法。
    BACKGROUND: No topical and systemic treatment is proven to be effective on Cutaneous Macular Amyloidosis (CMA). The physical removal of the deposited protein by lasers may be a better choice.
    OBJECTIVE: To compare the efficacy of different lasers including Pulsed dye laser (PDL), 1064 mode of Q-Switched Nd-YAG, CO2, and combined CO2 and Q-Switched Nd:YAG lasers for the treatment of CMA.
    METHODS: 17 adult female patients with clinical CMA and histopathological confirmation were included in this study. Four close 1 by 1 cm2 areas were treated simultaneously with PDL, Q-Switched Nd-YAG, CO2 and combined CO2 and Nd-YAG Lasers. The energy used for 585 nm PDL were 9 J/cm2 with the spot size of 5 mm. The energy used for 1064 nm mode of Nd-YAG laser was 5 Joules/cm2, with the spot size of 4 mm, and the frequency of 10 hertz. The energies used for CO2 laser were 4 and 3.5 Micro Joules for the first and second Passes.
    RESULTS: No desirable result obtained after PDL therapy. The Q-Switched Nd-YAG was effective in only 3 patients. All areas treated with CO2 laser were cleared.
    CONCLUSIONS: The CO2 laser is seems to be the most effective laser treatment of CMA.
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  • 文章类型: Clinical Trial, Phase II
    UNASSIGNED: This aim of this study was to determine the effect of 1540-nm nonablative fractional erbium on macular amyloidosis.
    UNASSIGNED: This phase-II clinical trial study has been performed with parallel group with blinding of the evaluator. The skin lesions of the patients (15 patients and 30 lesions) with cutaneous macular amyloidosis were randomly assigned into laser and no-treatment groups. In the laser group, treatment was performed by 1540-nm nonablative fractional erbium laser. Thereafter, the patients\' lesions were compared in terms of pigmentation, rippling, thickness, and subjective response.
    UNASSIGNED: The lesions of the intervention group significantly improved in the three-month follow-up compared to the control group (in the control and intervention group, improved pigmentation was observed in 20 and 53.3% with p = .02, improved rippling in 6.7 and 60% with p = .007, diminished lichenification in 0 and 53.1% with p = .007, and overall lesion improvement in 20 and 60% with p = .03, respectively). In investigating the subjective response through patient global assessment, the patients in the intervention group had a greater satisfaction (p = .01). There was a considerable improvement of pruritus in the intervention group (p = .001).
    UNASSIGNED: Use of 1540-nm nonablative fractional erbium laser offered a suitable efficacy to treat macular amyloidosis without significant complications.
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  • 文章类型: Journal Article
    UNASSIGNED: Primary cutaneous amyloidosis (PCA) can be classified into four principal categories: macular amyloidosis, lichen amyloidosis, biphasic, and nodular amyloidosis. Some unusual variants such as widespread diffuse hyperpigmentation without papules, poikiloderma like involvement, lesions following Blaschko\'s line, etc., have also been reported. However, not much data are available regarding the demography, epidemiology, clinical patterns, and distribution and histopathological findings, especially from the eastern part of India.
    UNASSIGNED: We conducted a cross-sectional, institution-based study to evaluate clinicopathological pattern and factors of PCA in eastern India.
    UNASSIGNED: We recorded clinical and histopathological findings of 100 consecutive patients of PCA presenting to a tertiary care institution of Kolkata in eastern India.
    UNASSIGNED: We found female patients of PCA outnumber male (M:F =1:1.9) with majority of patients being young adults (56%) between 20 and 40 years of age. More than half (54%) of the patients were pruritic. The severity of pruritus is significantly more associated with lichenoid and biphasic variants over macular amyloidosis. Positive family history was recorded in 17% of cases. Macular variant was the most common variant constituting 48% of the total PCA. We also found that the association with history of friction and scrubbing and photo-exposure were statistically insignificant. However, duration of the disease has statistically significant association with the disease morphology. Congo red stain showed these deposits as reddish orange substance in 28 patients out of 64 patients\' samples on which Congo red could be performed.
    UNASSIGNED: Our study revealed that many concepts of pathogenesis of PCA including friction and photoexposure might have lesser importance. However, morphological types were significantly associated with the duration of the disease and symptom severity.
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