使用新型静脉注射免疫球蛋白制剂治疗复杂的硬肌水肿病例

IF 0.9 Q4 DERMATOLOGY Case Reports in Dermatology Pub Date : 2024-08-27 eCollection Date: 2024-01-01 DOI:10.1159/000540519
Anastasia Sophie Vollmer, Nadine Wiedenmayer, Julia K Winkler, Alexander H Enk
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引用次数: 0

摘要

导言:我们报告了一例接受大剂量静脉注射免疫球蛋白(IVIgs)治疗的硬肌水肿患者的病例,该患者在改用新型 IVIg 制剂 "Yimmugo "后,疲劳减轻,头痛减少。硬化性粘液性水肿是一种罕见的慢性皮肤粘液病,常伴有单克隆丙种球蛋白病和全身并发症,可能危及生命。该病的特征是皮肤丘疹性糜烂和硬化。由于发病机制不明确,硬皮水肿的治疗具有挑战性。大剂量静脉注射是改善皮肤表现的一种很有前景的治疗方法:本病例是一名 57 岁的硬肌水肿和单克隆丙种球蛋白病患者,终末期肾衰竭,接受血液透析治疗(每周 3 次)。通过每公斤体重 2 克、每 3 周注射 2 天的大剂量 IVIg 治疗,患者的皮肤症状最终得到稳定。然而,疾病的稳定是以流感样副作用为代价的,这些副作用严重影响了患者的日常生活。在转用 Yimmugo® 后,患者的生活质量有所改善:结论:要最终评估这种新型 IVIg 制剂的有效性和耐受性,必须进行进一步的随访。
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Treatment of a Complex Case of Scleromyxedema Using a Novel Intravenous Immunoglobulin Preparation.

Introduction: We provide a case report on a patient with scleromyxedema treated with high-dose intravenous immunoglobulins (IVIgs) who reported less fatigue and fewer headaches after transitioning to Yimmugo, a novel IVIg preparation. Scleromyxedema is a rare and chronic cutaneous mucinosis, frequently associated with monoclonal gammopathy and systemic complications, which may be life-threatening. The disease is characterized by papular eruptions and sclerosis of the skin. Treatment of scleromyxedema is challenging because of its unclear pathogenesis. High-dose IVIgs are a promising treatment option to improve cutaneous manifestations.

Case presentation: We present the case of a 57-year-old patient with scleromyxedema and monoclonal gammopathy with end-stage renal failure on hemodialysis (3 times a week). Stabilization of skin symptoms was finally achieved by high-dose IVIg therapy administered at 2 g per kg bodyweight distributed over 2 days every 3 weeks. However, disease stabilization came at the expense of flu-like side effects that significantly affected daily life. After transitioning to Yimmugo®, the patient reported an improved quality of life.

Conclusion: Further follow-up is essential to conclusively evaluate effectiveness and tolerability of this novel IVIg preparation.

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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
期刊最新文献
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