keratosis pilaris

  • 文章类型: Journal Article
    阻断BCR-ABL活性的酪氨酸激酶抑制剂(TKIs)的出现彻底改变了慢性粒细胞白血病(CML)患者的治疗管理。不良皮肤反应(ACRs)是与使用BCR-ABLTKIs相关的常见非血液学不良事件。在用这些药物治疗的患者中,已经描述了类似毛发角化病(KP)的喷发特征模式,尤其是尼洛替尼和达沙替尼。这种ACR的发病机制仍然未知。这种类型的反应似乎是罕见的伊马替尼。这里,我们报告了一个无症状的KP样喷发的老年患者,伊马替尼治疗CML1个月后出现.病例报告伴随着对BCR-ABL抑制剂治疗的CML患者的类似反应的回顾,试图对此类药物的分子靶标和这种ACR的可能机制进行概述。
    The advent of tyrosine kinase inhibitors (TKIs) blocking BCR-ABL activity has revolutionized the therapeutic management of patients with chronic myeloid leukemia (CML). Adverse cutaneous reactions (ACRs) are common nonhematologic adverse events associated with the use of BCR-ABL TKIs. A characteristic pattern of eruption resembling keratosis pilaris (KP) has been described in patients treated with these drugs, especially nilotinib and dasatinib. The pathogenesis of this ACR is still unknown. This type of reaction appears to be uncommon with imatinib. Here, we report the case of an elderly patient with an asymptomatic KP-like eruption, which appeared one month after starting treatment with imatinib for CML. The case presentation is accompanied by a review of similar reactions in patients with CML treated with BCR-ABL inhibitors, attempting to make an excursus on the molecular targets of such drugs and possible mechanisms underlying this ACR.
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  • 文章类型: Case Reports
    萎缩性毛发角化病(KPAF)是一种罕见的,世袭,毛囊疾病分类为毛发角化病(KP)的萎缩性亚型。如今,它可以用光和激光设备进行处理。波长<600nm的激光器,尤其是脉冲染料激光器(PDL),对KPAF的治疗有效。这里,我们提出了一个用585nm二极管激光治疗KPAF的例子,一种具有差分波长修改的光泵浦半导体(D-WMOPS)技术的激光系统。我们的病例是文献中报道的第一位使用这种激光技术治疗的患者。
    Keratosis pilaris atrophicans faciei (KPAF) is a rare, hereditary, follicular disorder categorized in the atrophicans subtypes of keratosis pilaris (KP). Nowadays it can be treated with light and laser devices. Lasers with wavelengths <600 nm, especially pulsed dye laser (PDL), are effective for treatments of KPAF. Here, we present a case with KPAF treated with 585 nm diode laser, a kind of laser system functioning with differential wavelength modified optically pumped semiconductor (D-WMOPS) technology. Our case is the first patient reported to have been treated with this laser technology in the literature.
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  • 文章类型: Journal Article
    疾病严重程度评估工具在评估皮肤病学的皮肤状况中起着很大的作用。目前,目前尚无经过验证的毛发角化病(KP)评估工具,良性但高度流行的卵泡疾病。一系列提出的评分工具已在不同的临床试验中用于评估KP的潜在治疗。对用于KP的当前评分系统的文献综述表明,与使用不同版本的未经验证的研究者全球评估(IGA)评分和四分位数评分系统的大多数研究缺乏一致性。对这些研究的回顾表明,目前在临床试验中评估KP的方法是主观的,不可靠,和不一致。标准化和验证的评分系统将是重要的,因为它可以用于临床试验以提高KP的当前知识。
    Disease severity assessment tools play a large part in evaluating skin conditions in dermatology. Currently, there is no existing validated assessment tool for keratosis pilaris (KP), a benign yet highly prevalent follicular disorder. A range of proposed scoring tools have been used in different clinical trials for the assessment of potential treatments for KP. A literature review of the current scoring systems used for KP shows that there is a lack of consistency with most studies using varying versions of unvalidated investigator global assessment (IGA) scores and quartile grading systems. A review of these studies shows that current methods of evaluating KP in clinical trials are subjective, unreliable, and inconsistent. A standardised and validated scoring system would be significant as it could be used in clinical trials to advance the current knowledge of KP.
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  • 文章类型: Journal Article
    毛囊角化病(KP)是一种常见的毛囊角化病,其特征是角化性毛囊丘疹伴不同程度的毛囊周围红斑。毛发角化病影响多达一半的正常儿童和多达四分之三的特应性皮炎儿童。KP在青春期突出,在老年人中较少见,但它可能发生在所有年龄段的儿童和成人。在这份报告中,我们描述了一例已知患有CHARGE综合征的13岁男孩,他在注射睾酮后出现全身性毛发角化病.据我们所知,这是第一例报道的由睾酮注射引起的全身性毛发角化病。
    Keratosis pilaris (KP) is a common disorder of follicular keratinization characterized by keratotic follicular papules with varying degrees of perifollicular erythema. Keratosis pilaris affects up to half of normal children and up to three-quarters of children with atopic dermatitis. KP is prominent during adolescence and less common in older people, but it may occur in children and adults of all ages. In this report, we describe the case of a 13-year-old boy known to have CHARGE syndrome who developed generalized keratosis pilaris after testosterone injections. To the best of our knowledge, this is the first reported case of generalized keratosis pilaris induced by testosterone injection.
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  • 文章类型: Journal Article
    目的:关于超重和肥胖儿童和青少年的皮肤特异性发现知之甚少。这项研究评估了皮肤体征与关键的营养和内分泌学参数的关联及其对肥胖年轻人生活质量的影响。
    方法:最初招募到三级医院的体重控制计划的所有患者都参加了这一跨学科研究,单中心,横断面研究。所有参与者都接受了详细的皮肤病学检查,人体测量和实验室检查。使用经过验证的问卷评估生活质量。
    结果:共有103名儿童和青少年(年龄11.6±2.5岁,41%女性,25%的青春期前,BMISDS2.6±0.5,HOMA评分3.3±4.2;平均值±SD)在12个月的研究期内招募。皮肤影响与BMI增加和年龄增加呈线性相关。最常见的皮肤发现是[%]条(71.0),毛发角化病(64.7),黑棘皮病(45.0),寻常痤疮(39.2),顶索(25.5)和足底角化过度(17.6)。HOMA评分与黑棘皮病相关(p=0.047),毛发角化病(p=0.019)和寻常痤疮(p<0.001)。一般平均生活质量评分,根据WHO-5的评估,100人中有70人。总共38.9%的参与者报告皮肤病学QoL受损。
    结论:这项研究表明儿童和青少年肥胖患者皮肤病变的患病率很高。皮肤损伤和HOMA评分之间的关联表明皮肤表现是胰岛素抵抗的标志。为了预防继发性疾病和提高生活质量,彻底的皮肤检查和跨学科合作是必要的。
    UNASSIGNED: Little is known about specific cutaneous findings in children and adolescents with overweight and obesity. This study assessed the association of skin signs with pivotal auxological and endocrinological parameters and their influence on the quality of life (QoL) of young people with obesity.
    UNASSIGNED: All patients initially recruited for a tertiary hospital\'s weight control program were offered participation in this interdisciplinary, single-center, cross-sectional study. All participants underwent a detailed dermatological examination, anthropometric measurements and laboratory examinations. QoL was assessed with validated questionnaires.
    UNASSIGNED: A total of 103 children and adolescents (age 11.6 ±2.5 years, 41% female, 25% prepubertal, BMI SDS 2.6 ± 0.5, homeostatic model assessment (HOMA) score 3.3 ± 4.2; mean ± s.d.) were recruited in a 12-month study period. Skin affections were linearly associated with increasing BMI and higher age. The most common skin findings were (%) striae distensae (71.0), keratosis pilaris (64.7), acanthosis nigricans (45.0), acne vulgaris (39.2), acrochordons (25.5) and plantar hyperkeratosis (17.6). The HOMA score was associated with acanthosis nigricans (P = 0.047), keratosis pilaris (P = 0.019) and acne vulgaris (P < 0.001). The general mean QoL(QoL) score, as assessed by the WHO-5, was 70 out of 100. A total of 38.9% of participants reported impaired dermatological QoL.
    UNASSIGNED: This study shows the high prevalence of skin lesions in children and adolescents with obesity. The association between skin lesions and the HOMA score indicates that skin manifestations are a marker of insulin resistance. To prevent secondary diseases and improve QoL, thorough skin examinations and interdisciplinary cooperation are necessary.
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  • 文章类型: Journal Article
    放射病是由属于RAS/MAPK通路的基因中的种系致病变异引起的罕见遗传疾病,它发出细胞增殖信号,分化,生存和死亡。这种信号通路的功能障碍导致具有重叠临床表现的综合征。皮肤和附件病变是放射病的主要临床体征,比如心脏皮肤综合征,努南综合征伴多个腹水,以前被称为LEOPARD综合征,科斯特洛综合征,神经纤维瘤病(NF1),Legius综合征,Noonan样综合征伴毛发疏松(NSLH)和Noonan综合征。作为NF1,最常见的放射病之一,1882年描述,其临床特征很好地描述,我们将专注于皮肤病学诊断,非NF1神经病的管理和护理,这是鲜为人知和最近描述。皮肤病学表现是重要的临床诊断要素,可以帮助在放射病之间进行鉴别诊断。它们可以影响真皮和表皮,导致色素性病变(黑素细胞痣,咖啡厅-au-lait斑点,和lentigines),角化过度(毛发角化病,光敏性红斑,和掌plant角化病)或增生。迄今为止,与恶性肿瘤有罕见的已知联系,但皮肤损伤往往需要密切关注,因为它们会严重影响生活质量。
    RASopathies are rare genetic disorders caused by germline pathogenic variants in genes belonging to the RAS/MAPK pathway, which signals cell proliferation, differentiation, survival and death. The dysfunction of such signaling pathway causes syndromes with overlapping clinical manifestations. Skin and adnexal lesions are the cardinal clinical signs of RASopathies, such as cardiofaciocutaneous syndrome, Noonan syndrome with multiple lentigines, formerly known as LEOPARD syndrome, Costello syndrome, neurofibromatosis (NF1), Legius syndrome, Noonan-like syndrome with loose anagen hair (NSLH) and Noonan syndrome. As NF1, one of the most common RASopathies, described in 1882, has its clinical features well delineated, we will focus on the dermatological diagnosis, management and care of non-NF1 RASopathies, which are less known and more recently described. Dermatological manifestations are important clinical diagnostic elements that can aid differential diagnosis among RASopathies. They can affect dermis and epidermis, causing pigmented lesions (melanocytic nevi, café-au-lait spots, and lentigines), hyperkeratosis (keratosis pilaris, ulerythema ophryogenes, and palmoplantar keratosis) or hyperplasia. To date there are rare known links to malignancy, but oftentimes skin lesions require close attention because they can highly affect quality of life.
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  • 文章类型: Case Reports
    We report a case of a 13-year-old boy who presented with eruptive monomorphic white papules on the trunk and arms involving regions previously affected by toxic epidermal necrolysis (TEN). Biopsy revealed compact keratin involving the hair follicle and sparse mixed perivascular infiltrate, findings consistent with lichen spinulosus. Improvement was noted after treatment with ammonium lactate 12% lotion. While cutaneous dyschromia and xerosis are common after TEN, lichen spinulosus has not yet been described in the literature. It is important for providers to be aware of any potential cutaneous sequelae of TEN that can affect quality of life in order to best counsel their patients.
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  • 文章类型: Case Reports
    原发性红斑(UO)或毛发角化病是一种主要影响面部的角化疾病。UO中的炎症过程可能最终导致脱发。这种疾病的发病率仍然未知。我们在一名28岁的男性中介绍了一例UO,这是沙特阿拉伯的第一个。
    Ulerythema ophryogenes (UO) or keratosis pilaris rubra atrophicans faciei is a disorder of keratinization that primarily affects the face. The inflammatory process in UO may eventually result in alopecia. The incidence of this disorder is still unknown. We present a case of UO in a 28-year-old male, the first of its kind in Saudi Arabia.
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  • 文章类型: Journal Article
    暂无摘要。
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  • 文章类型: Journal Article
    背景:毛发角化病(KP)是一种毁容性疾病,对治疗有抵抗力。有几种治疗方法,但疗效有限。这个未来,评估者失明,分体对比研究探讨了长脉冲755nm翠绿宝石激光治疗KP的疗效和安全性。
    方法:本研究纳入了22例双侧臂KP患者。所有参与者都被随机分配,并在间隔3周的四个疗程中,在左或右臂上使用长脉冲755nm的翠绿宝石激光治疗。统一的保湿乳液每天一次施用于左臂和右臂。记录医师评估得分和患者自我评估得分,皮肤镜检查的皮肤影像学改变,高频超声,在基线和第四次治疗后4周获得皮肤活检。
    结果:在完成研究的21名患者中,15人是女性,6人是男性。在第四次治疗后4周,激光侧显示出明显较低的总量(2.0比4.5),粗糙度(1.0对2.0),根据医生的评估,红肿(1.0对2.0)评分(所有P<0.05)。此外,激光侧显示出明显较低的总量(2.0对4.0),粗糙度(1.0对2.0),根据患者的自我评估,红肿评分(1.0对2.0)(均P<0.05)。通过皮肤镜和超声检查获得卵泡塞改善的患者比例(57.1%对14.3%),毛囊周围红斑(52.4%对9.5%),卵泡周色素沉着过度(47.6%对14.3%),激光侧的表皮凸起数量(57.1%对19.1%)明显高于对照组(均P<0.05)。组织病理学显示,末次访视时滤泡塞和炎性细胞浸润得到改善。三名患者表现出可逆性的炎症后色素沉着过度。
    结论:长脉冲755nm翠绿宝石激光治疗对KP皮肤粗糙和发红均有效且安全。
    背景:ChiCTR2100054489。
    BACKGROUND: Keratosis pilaris (KP) is a disfiguring disease and is resistant to treatment. Several treatment methods are available, but the efficacy is limited. This prospective, rater-blinded, split-body comparative study investigated the efficacy and safety of long-pulsed 755-nm alexandrite laser in the treatment of KP.
    METHODS: Twenty-two patients with KP of bilateral arms were enrolled in this study. All participants were randomized and treated with a long-pulsed 755-nm alexandrite laser on the left or right arm in four sessions held 3 weeks apart. The unified moisturizing lotion was applied on both left and right arms once a day. Physicians\' assessment scores and patients\' self-assessment scores were recorded, and skin imaging changes in dermoscopy, high-frequency ultrasound, and skin biopsy were obtained at baseline and 4 weeks after the fourth treatment.
    RESULTS: Of the 21 patients who completed the study, 15 were women and 6 were men. At 4 weeks after the fourth treatment, the laser side showed significantly lower total (2.0 versus 4.5), roughness (1.0 versus 2.0), and redness (1.0 versus 2.0) scores according to physicians\' assessment (all P < 0.05). Furthermore, the laser side showed significantly lower total (2.0 versus 4.0), roughness (1.0 versus 2.0), and redness scores (1.0 versus 2.0) according to the patients\' self-assessment (all P < 0.05). The proportions of patients who achieved dermoscopically and ultrasonographically showed excellent improvements in follicular plugs (57.1% versus 14.3%), perifollicular erythema (52.4% versus 9.5%), perifollicular hyperpigmentation (47.6% versus 14.3%), and the number of epidermal bulges (57.1% versus 19.1%) in the laser side was significantly higher than those who achieved such improvements in the control side (all P < 0.05). Histopathology showed that the follicular plugs and inflammatory cell infiltration were improved at the final visit. Three patients exhibited reversible postinflammatory hyperpigmentation.
    CONCLUSIONS: Long-pulsed 755-nm alexandrite laser treatment is effective and safe in treating both skin roughness and redness in KP.
    BACKGROUND: ChiCTR2100054489.
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