嗜酸性粒细胞筋膜炎:揭开罕见病例的临床面纱和文献综述。

IF 1.2 4区 医学 Q4 DEVELOPMENTAL BIOLOGY
Aifer Cherim, Alexandra Eugenia Bastian, Cristiana Gabriela Popp, Maia Ioana Mihon, Ion Cristian Efrem, Ananu Florentin Vreju, Răzvan Adrian Ionescu
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引用次数: 0

摘要

嗜酸性粒细胞筋膜炎(EF)因其罕见性和与硬皮病的相似性,仍然是诊断上的一个难题。本病例报告旨在深入探讨嗜酸性粒细胞性筋膜炎的临床细微差别,强调准确诊断和有效治疗的重要性。一名 52 岁的男性在本诊所就诊前四个月出现双侧前臂和小腿变硬,并伴有红斑、瘙痒和疼痛。症状最初出现在双侧前臂,后来发展到小腿、手臂远端和大腿。临床检查发现,前臂和小腿出现对称性斑块,红斑、色素沉着和色素减退,向近端延伸,"凹槽征 "阳性,膝关节屈曲中度困难。尽管有这些发现,但患者总体状况良好,没有其他明显的临床症状。初步实验室检查结果显示,嗜酸性粒细胞水平略有升高,C反应蛋白(CRP)升高,红细胞沉降率(ESR)正常,抗核抗体和硬皮病特异性抗体阴性。磁共振成像(MRI)显示筋膜信号增强且增厚,筋膜肌肉活检显示明显水肿和炎性淋巴浆细胞浸润,与 EF 诊断一致。患者对全身皮质类固醇的反应良好。EF主要影响30至60岁的男性,其特点是发病突然,病因不明。鉴别诊断需要仔细排除硬皮病和其他类似疾病。皮肤肌肉活检和核磁共振成像等诊断方法可发现炎症浸润和筋膜增厚等特征性结果。准确诊断和与硬皮病的鉴别至关重要,使用糖皮质激素和免疫抑制剂进行早期干预可改善长期预后。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Eosinophilic fasciitis: unraveling the clinical tapestry of a rare case and review of literature.

Eosinophilic fasciitis (EF) remains a diagnostic challenge due to its rarity and resemblance to scleroderma. This case report aims to provide a cohesive exploration of EF's clinical nuances, emphasizing the importance of accurate diagnosis and effective management. A 52-year-old male developed bilateral forearm and calf hardening, along with erythema, pruritus, and pain four months prior to the presentation in our Clinic. The symptoms initially debuted bilaterally in the forearms and progressed to involve the calves, distal arms, and thighs. Clinical examination revealed symmetrical plaques on forearms and calves, featuring erythematous, hyper, and hypopigmented elements extending proximally, a positive "groove sign" and a moderate difficulty in knee joint flexion. Despite these findings, the patient was generally in good condition, without any other notable clinical signs. Initial laboratory findings showed slightly increased percentual eosinophil levels, elevated C-reactive protein (CRP), normal erythrocyte sedimentation rate (ESR), and negative antinuclear and scleroderma specific antibodies. Magnetic resonance imaging (MRI) demonstrated enhanced fascial signal and thickening while the fascia-muscle biopsy revealed marked edema and inflammatory lymphoplasmacytic infiltrate, consistent with the diagnosis of EF. The patient showed a favorable response to systemic corticosteroids. EF predominantly affects males aged 30 to 60 and is characterized by a sudden onset and unclear etiological factors. Differential diagnosis requires careful exclusion of scleroderma and other mimicking conditions. Diagnostic modalities such as skin-muscle biopsy and MRI reveal characteristic findings like inflammatory infiltrate and fascial thickening. Accurate diagnosis and differentiation from scleroderma are crucial, with early intervention involving glucocorticoids and immunosuppressive agents improving long-term outcomes.

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来源期刊
CiteScore
1.70
自引率
20.00%
发文量
221
审稿时长
3-8 weeks
期刊介绍: Romanian Journal of Morphology and Embryology (Rom J Morphol Embryol) publishes studies on all aspects of normal morphology and human comparative and experimental pathology. The Journal accepts only researches that utilize modern investigation methods (studies of anatomy, pathology, cytopathology, immunohistochemistry, histochemistry, immunology, morphometry, molecular and cellular biology, electronic microscopy, etc.).
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