Association between Localized Scleroderma Cutaneous Assessment Tool and clinicopathologic characteristics in patients with morphea

Y. Gürsel Ürün, E. Usturalı Keskin
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Abstract

Background and Design: Morphea is also known as localized scleroderma. It is a rare autoimmune skin disease characterized by inflammation and sclerosis in the dermis and sometimes in the subcutaneous tissue. Laboratory findings, imaging, and histopathological features facilitate diagnosis and provide sufficient information about disease severity. Clinicopathologic correlations and severity factors in morphea are poorly described. Thus, this study aimed to review the clinical and histopathological features and treatment responses of patients with morphea and compare these features with disease activity and damage scores to identify new tools for assessing disease severity other than clinical findings. The applicability of the Localized Scleroderma Cutaneous Assessment Tool in clinical practice was also evaluated. Materials and Methods: This study reviewed data of 41 patients who had a histopathologically confirmed diagnosis of morphea and had been followed up regularly for at least 6 months. The modified Localized Scleroderma Skin Severity Index (mLoSSI), Localized Scleroderma Skin Damage Index (LoSDI), Physician Global Assessment-Activity (PGA-A), and Physician Global Assessment-Damage (PGA-D) were calculated at baseline and final treatment. Results: Among morphea subtypes, superficial morphea had significantly more sclerosis in the papillary dermis and plaque-type morphea had significantly more sclerosis in the reticular dermis (p<0.05). When positive antinuclear antibody (ANA) and high levels of thyroid autoantibodies were compared with mLoSSI, LoSDI, PGA-A, and PGA-D scores at baseline, no significant correlation was found. Comparison of the subgroups according to the initial mLoSSI and LoSDI scores revealed no significant histopathological differences between the groups. Conclusion: Although the mLoSSI, LoSDI, PGA-A, and PGA-D scores can be successfully used for the follow-up and treatment of patients with morphea, no correlation was found between positive ANA, high levels of thyroid autoantibodies, and histopathological features. autoimmune diseases, clinical symptoms, and concomitant lichen sclerosus et atrophicus (LSA) were recorded. Laboratory results, detailed treatment modalities, treatment duration, and treatment responses were assessed. The same dermatologist performed a biopsy of the erythematous rim of the inflammatory lesions or central sclerosis of the lesions that did not clinically show inflammation using a 4 mm punch biopsy instrument. Incisional biopsy was performed in patients with a provisional diagnosis of linear morphea, deep type morphea, or eosinophilic fasciitis.
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局限性硬皮病皮肤评估工具与变形患者临床病理特征的相关性
背景与设计:多发性硬皮病又称局部硬皮病。这是一种罕见的自身免疫性皮肤病,其特征是真皮层炎症和硬化症,有时在皮下组织。实验室检查、影像学和组织病理学特征有助于诊断,并提供有关疾病严重程度的充分信息。morphea的临床病理相关性和严重程度因素描述甚少。因此,本研究旨在回顾morphea患者的临床和组织病理学特征和治疗反应,并将这些特征与疾病活动性和损伤评分进行比较,以确定除临床表现外评估疾病严重程度的新工具。并对局限性硬皮病皮肤评估工具在临床实践中的适用性进行了评价。材料和方法:本研究回顾了41例经组织病理学确诊为睡眠不足的患者的资料,并定期随访至少6个月。在基线和最终治疗时计算改良的局部硬皮病皮肤严重程度指数(mLoSSI)、局部硬皮病皮肤损伤指数(LoSDI)、医生整体评估-活动(PGA-A)和医生整体评估-损伤(PGA-D)。结果:在morphea亚型中,浅表性morphea明显多于乳头状真皮硬化,斑块型morphea明显多于网状真皮硬化(p<0.05)。当抗核抗体(ANA)阳性和高水平甲状腺自身抗体与基线时的mLoSSI、LoSDI、PGA-A和PGA-D评分进行比较时,未发现显著相关性。根据初始mLoSSI和LoSDI评分对亚组进行比较,结果显示各组之间没有明显的组织病理学差异。结论:虽然mLoSSI、LoSDI、PGA-A、PGA-D评分可以成功用于吗啡患者的随访和治疗,但ANA阳性、甲状腺自身抗体水平高与组织病理学特征之间没有相关性。记录自身免疫性疾病、临床症状和伴随的硬化性萎缩性地衣(LSA)。评估了实验室结果、详细的治疗方式、治疗时间和治疗反应。同一位皮肤科医生使用4毫米穿孔活检仪对炎性病变的红斑边缘或没有临床炎症表现的病变的中央硬化进行活检。对暂时诊断为线状脑膜瘤、深部脑膜瘤或嗜酸性筋膜炎的患者进行切口活检。
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来源期刊
CiteScore
0.30
自引率
0.00%
发文量
52
审稿时长
6-12 weeks
期刊最新文献
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