硬黏液水肿的临床特点和治疗结果:一项10年回顾性调查。

IF 0.9 Q4 DERMATOLOGY Case Reports in Dermatology Pub Date : 2022-06-27 eCollection Date: 2022-05-01 DOI:10.1159/000525211
Abtin Ansari, Zohre Erfani, Maryam Daneshpazhooh, Leila Mahmoudi, Zahra Saffarian, Kambiz Kamyab, Hamidreza Mahmoudi
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引用次数: 0

摘要

硬化黏液性水肿是一种罕见但重要的皮肤黏液病,表现为皮肤学表现,如蜡样丘疹、弥漫性硬结,以及非皮肤学表现,如神经和肾脏疾病。我们报告了14例诊断为硬黏液水肿的患者的特征和治疗及其随访的病例系列数据。患者根据硬黏液性水肿的诊断标准进入研究。还记录了合并症,以评估其对治疗过程的影响。分别评估临床病理和实验室结果以及对其治疗的反应。静脉注射免疫球蛋白(IVIG)后有显著改善。尽管这种情况缺乏明确的治疗方法,但目前的研究表明,IVIG的应用可以改善皮肤和全身症状。尤其是年轻患者,对IVIG的使用反应显著。IVIG治疗硬黏液性水肿的潜在疗效有待进一步研究。
本文章由计算机程序翻译,如有差异,请以英文原文为准。

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Clinical Characteristics and Treatment Outcomes of Scleromyxedema: A 10-Year Retrospective Survey.

Scleromyxedema is a rare but important mucinosis disorder of the skin that is presented with dermatological manifestations such as waxy papules, diffuse induration, and nondermatologic involvements like neurological and renal disorders. We report a case series of the data regarding the characteristics and treatment of 14 patients diagnosed with scleromyxedema and their follow-up. Patients entered the study based on scleromyxedema diagnosis criteria. Comorbidities were also recorded to evaluate their effect on the treatment process. Clinicopathological and laboratory findings and responses to their treatment were evaluated separately. There was a significant improvement after administering intravenous immunoglobulin (IVIG). Despite the lack of a definite treatment for this condition, the present study shows that the application of IVIG can improve both cutaneous and systemic symptoms. Younger patients, in particular, responded significantly to the use of IVIG. More studies are required to investigate the potential efficacy of IVIG in the treatment of scleromyxedema.

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来源期刊
CiteScore
1.60
自引率
0.00%
发文量
57
审稿时长
9 weeks
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