Skin involvement

皮肤受累
  • 文章类型: Journal Article
    背景:经典霍奇金淋巴瘤(CHL)的皮肤受累是当今时代极为罕见的现象。迄今为止,文献中从未报道过霍奇金淋巴瘤皮肤受累的单个大病例系列。
    方法:在1990年至2020年之间,在不同机构中对指定为“皮肤”和“霍奇金”的病例进行了全面搜索。确定了25个病例,每例病例由至少3名经董事会认证的皮肤病理学家和/或血液病理学家独立审查.
    结果:所有病例均为全身性CHL伴继发性皮肤播散的例子。单个病变,通常是肿瘤,56%的病例出现结节或浸润斑块,28%的病例出现多发病变.大多数患者(86%-12/14)在首次诊断时被诊断为IV期疾病。HL的临床(首次)诊断与皮肤病变发展之间的间隔为6至108个月(平均33.75个月)。对这些病例(在最初诊断时)的综合组织病理学评估显示,在60%的病例(15/25)中诊断为未另外指定的经典HL(NOS)。24%(6/25)的结节性硬化症类型,混合细胞率为12%(3/25),淋巴细胞耗竭4%(1/25)。
    结论:我们提供了大量的CHL继发皮肤受累与CHL相关的文献,形态学,和免疫表型特征。
    BACKGROUND: Cutaneous involvement by classic Hodgkin lymphoma (CHL) is an extraordinarily rare phenomenon in the current era. To date, no single large case series of cutaneous involvement by Hodgkin lymphoma has ever been reported in the literature.
    METHODS: A comprehensive search for cases designated \"skin\" and \"Hodgkin\" was performed at different institutions between 1990 and 2020. Twenty-five cases were identified, and each case was independently reviewed by at least three board-certified dermatopathologists and/or hematopathologists.
    RESULTS: All cases represented examples of systemic CHL with secondary skin dissemination. A single lesion, usually a tumor, nodule or infiltrative plaque was observed in 56% of cases and multiple lesions were present in 28% of cases. Most patients (86%-12/14) had a diagnosis of stage IV disease at first diagnosis. The interval between the clinical (first) diagnosis of HL and the development of skin lesions ranged between 6 and 108 months (average 33.75 months). Comprehensive histopathologic evaluation of these cases (at the initial diagnosis) revealed a diagnosis of classic HL not otherwise specified (NOS) in 60% of cases (15/25), nodular sclerosis type in 24% (6/25), mixed cellularity in 12% (3/25), and lymphocyte depleted in 4% (1/25).
    CONCLUSIONS: We provide documentation of a large series of CHL with secondary skin involvement in association with CHL with additional clinical, morphologic, and immunophenotypic features.
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  • 文章类型: Case Reports
    Mantle cell lymphoma (MCL) is a rare type of non-Hodgkin lymphoma that commonly affects extranodal sites. The most commonly affected sites are the bone marrow, gastrointestinal tract, Waldeyer\'s ring, lung, and pleura. We report the case of an 80-year-old diabetic male, in MCL remission, who presented with a small dome-shaped nodule on his calf and an ipsilateral second digit non-healing ulceration after a traumatic fall. Despite surgical and conservative treatment, the wound worsened, resulting in histopathologic examination, which confirmed the presence of lymphocytes, indicating MCL relapse. This case was followed up for approximately 3 months until the patient died. Our case is an example of pedal manifestations of skin involvement of MCL, which, on consideration of the clinical manifestations also, can be confused with a nonhealing diabetic wound. The clinical significance of our case study is to assist in the diagnosis, management, and prognosis of a patient with MCL.
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  • 文章类型: Journal Article
    LcSSc is associated with ACAs and a mild course, whereas dcSSc is associated with anti-topoisomerase antibodies (ATAs) and a more severe course. However, ATAs are also present in lcSSc. Little is known about survival and organ involvement in this subgroup. The aim of this study is to determine whether survival and organ involvement of lcSSc ATA-positive patients differs from lcSSc ATA-negative or dcSSc ATA-positive patients. Furthermore, transition from lcSSc to dcSSc was evaluated.
    Data from The Nijmegen Systemic Sclerosis cohort were used, with up to 15 years of follow-up. Kaplan-Meier analysis was performed for survival and organ involvement, including interstitial lung disease, pulmonary arterial hypertension, cardiac involvement and Scleroderma Renal Crises. Cox proportional hazard modelling was performed to adjust for confounders.
    A total of 460 patients were included: 58 (13%) lcSSc ATA-positive patients, 237 (52%) lcSSc ATA-negative patients and 78 (17%) dcSSc ATA-positive patients. Cumulative survival in lcSSc ATA-positive patients was 75%, in lcSSc ATA-negative patients 58% and in dcSSc ATA-positive patients 53%. Interstitial lung disease was more prevalent in lcSSc ATA-positive patients (49%) than in lcSSc ATA-negative patients (25%), but less than in dcSSc ATA-positive patients (60%). Forty-eight patients developed dcSSc: 24 ATA-negative and 24 ATA-positive (P < 0.001).
    LcSSc ATA-positive patients differ from lcSSc ATA-negative patients and dcSSc ATA-positive patients concerning survival and organ involvement. LcSSc patients who are ATA-positive are more likely to develop dcSSc than lcSSc patients who are ATA negative.
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  • 文章类型: Journal Article
    OBJECTIVE: To investigate skin elasticity using acoustic radiation force impulse (ARFI) quantification in systemic sclerosis (SSc), and compare the modified Rodnan skin score (mRSS) with measured shear wave velocity (SWV) and thickness of the skin.
    METHODS: Fifteen patients with diffuse cutaneous SSc (dcSSc) and 15 age-matched and sex-matched healthy controls were evaluated. The SWV and thickness of skin were measured at 17 sites corresponding to those assessed in the mRSS in each participant. The SWV measurements of skin were compared between patients with dcSSc and healthy controls. The correlations between the mRSS and the skin SWV and thickness were explored using Spearman\'s correlation.
    RESULTS: The SWV values were higher in patients with dcSSc compared with healthy controls at right hand dorsum, right forearm, left hand dorsum, left forearm, right foot dorsum, and left foot dorsum (p < 0.05). In patients with dcSSc, the SWV values of uninvolved skin were higher than those of controls (p < 0.001), and the SWV values increased with increasing skin scores except for skin score 3 (p < 0.05). The sum of the SWV values correlated with total clinical skin score (r = 0.841, p < 0.001), and the sum of the skin thickness correlated with total clinical skin score (r = 0.740, p = 0.002).
    CONCLUSIONS: ARFI quantification is feasible and reliable for assessing the skin involvement in dcSSc. ARFI quantification could identify early skin change that may precede palpable skin involvement, and may be a valuable adjunct to skin evaluation in patients with SSc.
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