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解析药物反应伴嗜酸性粒细胞增多和全身症状(DRESS)的复杂性:来自意大利三级中心的病例系列的临床、实验室和组织病理学特征的见解。

Unraveling the complexities of drug reaction with eosinophilia and systemic symptoms (DRESS): Insights into clinical, laboratory, and histopathologic features of a case series from an Italian tertiary center.

机构信息

Unit of Allergology and Immunology, ASST Grande Ospedale Metropolitano Niguarda, Milan, Italy.

Vita-Salute San Raffaele University, Milan, Italy.

出版信息

Clin Dermatol. 2023 Nov-Dec;41(6):721-728. doi: 10.1016/j.clindermatol.2023.09.005. Epub 2023 Sep 18.

Abstract

Drug reaction with eosinophilia and systemic symptoms (DRESS) is a severe and potentially life-threatening drug hypersensitivity reaction. The diagnosis and management of DRESS are complicated due to its heterogeneous clinical and pathologic presentations, delayed onset of signs and symptoms, and unpredictable outcome. This retrospective study aimed to analyze cases of DRESS from a single Italian referring tertiary hospital center (Grande Ospedale Metropolitano Niguarda, Milan, Italy) with a focus on clinical features, causative drugs, histopathologic findings, and treatment. We have included 18 of 32 patients with a probable or definite diagnosis of DRESS. The study observed a slight predominance of women, with antimicrobials and allopurinol identified as the main causative drugs. Clinical manifestations varied, with a monomorphic maculopapular eruption being the most common, whereas facial edema and mucosal involvement were less frequently observed. Multiple organs were commonly affected, with liver and kidney involvement being prominent. Cardiac involvement was associated with the severity of eosinophilia. Laboratory evaluations showed elevated eosinophil levels and increased eosinophil cationic protein levels, supporting the role of eosinophils in DRESS pathogenesis. Histopathologic analysis revealed various patterns often coexisting in the same biopsy in 83% of cases, with interface dermatitis being the most frequent, followed by the perivascular pattern and the spongiotic/eczematous pattern. We observed eosinophils in the biopsy samples in about 50% of patients, and the relationship between peripheral eosinophilia and eosinophils in skin biopsies was not significant. In addition to the RegiSCAR score, age may play a role in predicting disease severity, as older patients with lower scores had poorer outcomes. The prognosis of DRESS depended on early identification, discontinuation of the causative agent, and appropriate therapy. Systemic corticosteroids were the primary treatment option.

摘要

药物反应伴嗜酸性粒细胞增多和全身症状(DRESS)是一种严重且潜在威胁生命的药物过敏反应。由于其临床表现和病理表现具有异质性、症状和体征出现时间延迟以及预后不可预测,DRESS 的诊断和管理较为复杂。本回顾性研究旨在分析来自意大利一家三级转诊医院(米兰的 Grande Ospedale Metropolitano Niguarda)的 32 例 DRESS 病例,重点关注临床特征、致病药物、组织病理学发现和治疗。我们纳入了 18 例可能或明确诊断为 DRESS 的患者。研究观察到女性略占优势,主要致病药物为抗生素和别嘌醇。临床表现多样,以单形性斑丘疹性皮疹最为常见,而面部水肿和黏膜受累则较少见。多个器官常受累,肝、肾受累较为突出。心脏受累与嗜酸性粒细胞增多的严重程度相关。实验室评估显示,嗜酸性粒细胞水平升高和嗜酸性粒细胞阳离子蛋白水平升高,支持嗜酸性粒细胞在 DRESS 发病机制中的作用。组织病理学分析显示,83%的病例在同一次活检中存在多种模式,其中界面性皮炎最为常见,其次是血管周围模式和海绵状/湿疹样模式。我们在约 50%的患者的活检样本中观察到嗜酸性粒细胞,外周嗜酸性粒细胞与皮肤活检中嗜酸性粒细胞之间的关系并不显著。除了 RegiSCAR 评分外,年龄可能在预测疾病严重程度方面发挥作用,因为评分较低的老年患者预后较差。DRESS 的预后取决于早期识别、停用致病药物和适当的治疗。全身皮质类固醇是主要的治疗选择。

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