IF 1.9 4区 医学 Q3 DERMATOLOGY Clinical, Cosmetic and Investigational Dermatology Pub Date : 2025-01-21 eCollection Date: 2025-01-01 DOI:10.2147/CCID.S492503
Aili Gao, Xin Tian, Dexiu Lang, Yue Chen
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摘要

硬皮粘液性水肿(Scleromyxedema,SM)是一种罕见的原发性皮肤粘液病,以全身性丘疹和硬皮病样表现为特征,常伴有单克隆丙种球蛋白病。本病例是一名 37 岁的男性 SM 患者,三年来颈部和背部出现淡黄色斑块。组织病理学检查发现粘蛋白沉积、成纤维细胞增生和纤维化,为诊断提供了支持。血清蛋白电泳发现了 IgG-λ 型副蛋白血症。值得注意的是,患者曾有乙型肝炎病毒(HBV)感染史,这可能与该病有关。口服曲安奈德治疗八周后,皮损明显好转。尽管 SM 的诊断标准已经确立,但人们对其发病机制仍然知之甚少。循环细胞因子的慢性抗原刺激和病毒感染(如 HBV)可能会导致疾病的发生。本病例突出了慢性 HBV 感染与 SM 之间的潜在联系,强调了进一步研究病毒病因的必要性。SM 在临床、组织学和全身方面均不同于硬皮病和巩膜水肿等相关疾病,因此需要仔细鉴别以进行适当的治疗。中国缺乏流行病学数据,也没有针对 SM 的标准化治疗指南,这凸显了临床医生在诊断和管理这种罕见疾病时所面临的挑战。本病例强调了多学科协作的重要性,尤其是在合并 HBV 等感染时。鉴于SM病程慢性、难以预测并可能累及全身,持续随访和警惕监测对优化患者预后至关重要。
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Unusual Cutaneous Manifestations in a Patient with a History of Hepatitis B: A Case of Scleromyxedema and Literature Review.

Scleromyxedema (SM) is a rare primary cutaneous mucinosis characterized by systemic papules and scleroderma-like manifestations, often associated with monoclonal gammopathy. We present the case of a 37-year-old male with SM who developed yellowish plaques on the neck and back over three years. Histopathological examination revealed mucin deposition, fibroblast proliferation, and fibrosis, supporting the diagnosis. Serum protein electrophoresis identified IgG-λ type paraproteinemia. Notably, the patient had a history of hepatitis B virus (HBV) infection, suggesting a potential association. Following eight weeks of oral triamcinolone acetonide therapy, the skin lesions improved significantly. Despite established diagnostic criteria for SM, its pathogenesis remains poorly understood. Chronic antigenic stimulation from circulating cytokines and viral infections, such as HBV, may contribute to disease development. This case highlights the potential link between chronic HBV infection and SM, emphasizing the need for further investigation into viral etiologies. SM differs clinically, histologically, and systemically from related disorders such as scleroderma and scleroedema, necessitating careful differentiation for appropriate management. The scarcity of epidemiological data and lack of standardized treatment guidelines for SM in China underscore the challenges clinicians face in diagnosing and managing this rare disease. This case reinforces the importance of multidisciplinary collaboration, particularly when co-infections such as HBV are present. Given the chronic, unpredictable course of SM and its potential systemic involvement, continuous follow-up and vigilant monitoring are essential to optimize patient outcomes.

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来源期刊
CiteScore
2.80
自引率
4.30%
发文量
353
审稿时长
16 weeks
期刊介绍: Clinical, Cosmetic and Investigational Dermatology is an international, peer-reviewed, open access journal that focuses on the latest clinical and experimental research in all aspects of skin disease and cosmetic interventions. Normal and pathological processes in skin development and aging, their modification and treatment, as well as basic research into histology of dermal and dermal structures that provide clinical insights and potential treatment options are key topics for the journal. Patient satisfaction, preference, quality of life, compliance, persistence and their role in developing new management options to optimize outcomes for target conditions constitute major areas of interest. The journal is characterized by the rapid reporting of clinical studies, reviews and original research in skin research and skin care. All areas of dermatology will be covered; contributions will be welcomed from all clinicians and basic science researchers globally.
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