Ansari Abtin, Erfani Zohre, Daneshpazhooh Maryam, Mahmoudi Leila, Saffarian Zahra, Kamyab Kambiz, Mahmoudi Hamidreza
Autoimmune Bullous Diseases Research Center, Tehran University of Medical Sciences, Tehran, Iran.
Department of Dermatopathology, Razi Hospital, Tehran University of Medical Sciences, Tehran, Iran.
Case Rep Dermatol. 2022 Jun 27;14(2):178-183. doi: 10.1159/000525211. eCollection 2022 May-Aug.
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.
硬化性黏液水肿是一种罕见但重要的皮肤黏蛋白沉积症,表现为皮肤症状,如蜡样丘疹、弥漫性硬结,以及非皮肤症状,如神经和肾脏疾病。我们报告了一系列关于14例诊断为硬化性黏液水肿患者的特征、治疗及随访数据。患者根据硬化性黏液水肿诊断标准纳入研究。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检查结果以及对治疗的反应。静脉注射免疫球蛋白(IVIG)后有显著改善。尽管这种疾病缺乏明确的治疗方法,但本研究表明IVIG的应用可改善皮肤和全身症状。特别是年轻患者,对IVIG的使用反应显著。需要更多研究来探讨IVIG治疗硬化性黏液水肿的潜在疗效。