macular amyloidosis

  • 文章类型: Journal Article
    背景:黄斑淀粉样变性是一种原发性局部皮肤淀粉样变性,表现为瘙痒性色斑,呈波纹状或网状。这项研究的目的是评估在黄斑淀粉样变性患者中使用局部氨甲环酸与微针刺的疗效。
    方法:本试验招募躯干或上肢双侧黄斑淀粉样变性患者。将所有患者的皮肤病变分为两部分,随机分为微针刺加氨甲环酸治疗组和仅微针刺治疗组。有四个疗程,间隔2周。由三名失明的皮肤科医生确定每组的色素沉着(基于照片和皮肤镜检查)和波纹改善的百分比。通过问卷测量患者满意度和瘙痒减少的水平,并定义为百分比。
    结果:20名女性被纳入本研究。患者的平均年龄(SD)为39.7(±10.13)岁。两组根据图像显示色素沉着的改善,皮肤镜,和涟漪图案。氨甲环酸组患者满意度为46.5%,单用微针治疗患者满意度为47.5%。然而,两组间差异无统计学意义(p值>0.05)。有趣的是,两组治疗4次后瘙痒改善61.66%.
    结论:微针刺是减少黄斑淀粉样变性瘙痒和色素沉着的合适方式。然而,局部应用氨甲环酸不会导致额外的改善。
    BACKGROUND: Macular amyloidosis is a form of primary localized cutaneous amyloidosis presented by pruritic pigmented macules in rippled or reticulate pattern. The aim of this study was to assess the efficacy of using topical tranexamic acid with micro-needling comparing to micro-needling alone in patients with macular amyloidosis.
    METHODS: Patients with bilaterally located macular amyloidosis on trunk or upper extremities were recruited in this trial. The skin lesions in all patients were divided into two parts which were randomly assigned to the group of treatment with micro-needling plus tranexamic acid and the group of micro-needling alone. There were four sessions of treatment with 2 weeks interval. The percentage of improvement in pigmentation (based on photographs and dermoscopy) and rippling of each group was determined by three blinded dermatologists. The level of patient satisfaction and reduction of pruritus was measured by a questionnaire and defined as a percentage.
    RESULTS: Twenty females were enrolled in this study. The mean (SD) patients\' age was 39.7 (±10.13) years. Both groups showed improvement in pigmentation based on images, dermoscopy, and rippling pattern. Patients\' satisfaction was 46.5% in tranexamic acid group and 47.5% in micro-needling alone. Nevertheless, there was no significant difference between both groups (p value >0.05). Interestingly, the pruritus improved 61.66% after four sessions of treatment in both groups.
    CONCLUSIONS: Micro-needling is a suitable modality for decreasing pruritus and pigmentation in macular amyloidosis. However, topical application of tranexamic acid does not lead to additional improvement.
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  • 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: Systematic Review
    黄斑淀粉样变性(MA)是一种原发性局部皮肤淀粉样变性,以淀粉样蛋白在乳头状真皮中沉积为特征。临床表现包括瘙痒性色素沉着斑和网状或波纹状斑点,主要发现在上背部和四肢。活检是确认MA的重要诊断工具。本系统综述集中于诊断为MA的患者的活检结果。
    Macular amyloidosis (MA) is a primary localized cutaneous amyloidosis, characterized by amyloid deposition in the papillary dermis. The clinical presentation includes pruritic hyperpigmented macules and patches with a reticulated or rippled pattern, primarily found on the upper back and extremities. Biopsy is an essential diagnostic tool for confirming MA. This systematic review focused on the biopsy outcomes in patients diagnosed with MA.
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  • 文章类型: Journal Article
    背景:原发性皮肤黄斑淀粉样变性(PCMA)是一种慢性瘙痒性皮肤病,其特征是皮肤中淀粉样蛋白的异质细胞外沉积。
    目的:本研究旨在评估外用6%加巴喷丁乳膏治疗PCMA患者的疗效。
    方法:在这项三盲临床试验中,共有34名患者,被诊断出患有PCMA,使用两种不同的策略,局部加巴喷丁作为活性组和载体乳膏作为对照组。
    结果:与基线值相比,两组的瘙痒评分降低均具有统计学意义(p<0.001)。研究1个月后,干预组的色素沉着评分明显低于对照组(p<0.001)。在第2个月(p=0.52)和第3个月(p=0.22),两组之间的色素沉着评分变化差异不显著。
    结论:这项研究的结果表明,外用加巴喷丁乳膏作为外用药物治疗PCMA相关瘙痒可能是有效的,没有任何明显的不良反应。建议在两种性别中进行更大的样本量和更长的持续时间的类似研究。
    BACKGROUND: Primary cutaneous macular amyloidosis (PCMA) is a chronic pruritic cutaneous disease characterized by heterogeneous extracellular deposition of amyloid protein in the skin.
    OBJECTIVE: This study aimed to evaluate the efficacy of topical 6% gabapentin cream for the treatment of patients with PCMA.
    METHODS: In this triple-blind clinical trial, a total of 34 patients, who were diagnosed with PCMA, treated using two different strategies of topical gabapentin as the active group and vehicle cream as the control group.
    RESULTS: Pruritus score reduction in both groups was statistically significant compared with the baseline value (p < 0.001). There was a significant pigmentation score reduction in intervention group compared with control group after 1 month of the study (p < 0.001). The differences of pigmentation score changes between the groups were not significant at month 2 (p = 0.52) and month 3 (p = 0.22).
    CONCLUSIONS: The results of this study suggest that topical gabapentin cream may be effective as a topical agent in the treatment of pruritus associated with PCMA without any significant adverse effects. It is recommended to perform similar studies with a larger sample size and longer duration in both sexes.
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  • 文章类型: Journal Article
    黄斑淀粉样变性(MA)是原发性局限性皮肤淀粉样变性(PLCA)的最常见类型之一,主要分布在躯干和四肢。由于广泛的治疗选择,本研究旨在比较1064nm调QNd:YAG激光和2940nmEr:YAG激光治疗MA的有效性。这项临床试验于2020-2021年对33名患有MA的女性进行。在每个病人中,病变被随机分为两个区域,A和B.A区进行了4个疗程,间隔4周的Q开关Nd:YAG激光1064nm。区域B以4周的间隔用Er:YAG激光器2940nm进行四次治疗。基础色素沉着程度及治疗后色素沉着程度,瘙痒强度,治疗前后,对患者和医生的满意度进行了测量和比较。研究后患者瘙痒症状明显改善(P<0.001),但两组之间在改善方面没有显着差异(P>0.05)。我们还发现两组患者在患者和医师满意度方面没有显着差异(P>0.05)。Q开关Nd:YAG激光和Er:YAG激光的使用导致瘙痒方面的改善,病人和医生的满意度,和完全改善病变的色素沉着。
    Macular amyloidosis (MA) is one of the most common types of primary localized cutaneous amyloidosis (PLCA), distributed predominantly over the trunk and extremities. Due to the vast therapeutic options, this study aims to compare the effectiveness of Q-switched Nd: YAG laser 1064 nm and Er: YAG laser 2940 nm in treating MA. This clinical trial was performed in 2020-2021 on 33 women with MA. In each patient, the lesion was randomly divided into two areas, A and B. Area A underwent four treatment sessions with 4-week intervals of Q-switched Nd: YAG laser 1064 nm. Area B underwent four treatment sessions with an Er: YAG laser 2940 nm at 4-week intervals. Degree of basal pigmentation and degree of pigmentation after treatment, pruritus intensity, before and after the treatment, and patient and physicians\' satisfaction were measured and compared. The pruritus in patients improved significantly after the study (P < 0.001), but no significant differences could be observed between the two groups regarding the improvements (P > 0.05). We also found no significant differences between the two groups of patients regarding patient and physicians\' satisfaction rates (P > 0.05). The use of both Q-switched Nd: YAG laser and Er: YAG laser resulted in improvements in terms of pruritus, patient and physicians\' satisfaction, and total improvement in pigmentation of the lesions.
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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: Journal Article
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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: Journal Article
    Macular amyloidosis (MA) is a common form of cutaneous amyloidosis that manifests as dark spots consisting of brown pigments with a rippled pattern on the skin, and the treatment of this condition is highly challenging. The aim of this study was to compare the efficacy and safety of intralesional injection of tranexamic acid (TXA) and topical application of Kligman combination drug in the treatment of macular amyloidosis. In this double-blind clinical trial, a total of 43 patients, who were diagnosed with MA, were treated with two different methods of intralesional injection of tranexamic acid and topical application of Kligman combination drug. Both therapeutic methods were effective in improving MA and significantly reduced hyperpigmentation of the treated areas, but tranexamic acid was significantly more effective than the Kligman combination drug. Significantly, greater improvements were observed in the group of patients treated with tranexamic acid. In the tranexamic acid treatment group, ΔE was reduced from 11.39 in the first session to 8.53 in the third session, and in the Kligman treatment group, it was reduced from 8.79 in the first session to 6.32 in the third session (p < 0.05). In addition, the pruritus score in patients treated with topical tranexamic acid injection was lower compared to the patients treated with the topical application of the Kligman combination drug. The results of this study demonstrated the significant positive effects of both treatment methods, but in terms of reducing melanin content, intralesional injection of tranexamic acid was a more effective method. Both treatments considered safe for MA. In tranexamic acid group, patients logically experienced a tolerable pain during injection but they significantly had significantly lower local pruritic discomfort during study. So, based on the positive findings of this study we suggest to use tranexamic acid in combination with other effective therapeutic methods for treatment of MA especially use of its topically applied form in combination with non-aggressive needling that results in better drug delivery without the experience of injection pain. Selection of the best administration route of tranexamic acid for hyperpigmented lesions depends on the each patient characteristic and their previous theraputic results that may vary case by case.
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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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