Anastasia Sophie Vollmer, Nadine Wiedenmayer, Julia K Winkler, Alexander H Enk
{"title":"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","authors":"Anastasia Sophie Vollmer, Nadine Wiedenmayer, Julia K Winkler, Alexander H Enk","doi":"10.1159/000540519","DOIUrl":null,"url":null,"abstract":"<p><strong>Introduction: </strong>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.</p><p><strong>Case presentation: </strong>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<sup>®</sup>, the patient reported an improved quality of life.</p><p><strong>Conclusion: </strong>Further follow-up is essential to conclusively evaluate effectiveness and tolerability of this novel IVIg preparation.</p>","PeriodicalId":9619,"journal":{"name":"Case Reports in Dermatology","volume":null,"pages":null},"PeriodicalIF":0.9000,"publicationDate":"2024-08-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11521433/pdf/","citationCount":"0","resultStr":"{\"title\":\"Treatment of a Complex Case of Scleromyxedema Using a Novel Intravenous Immunoglobulin Preparation.\",\"authors\":\"Anastasia Sophie Vollmer, Nadine Wiedenmayer, Julia K Winkler, Alexander H Enk\",\"doi\":\"10.1159/000540519\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"<p><strong>Introduction: </strong>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.</p><p><strong>Case presentation: </strong>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<sup>®</sup>, the patient reported an improved quality of life.</p><p><strong>Conclusion: </strong>Further follow-up is essential to conclusively evaluate effectiveness and tolerability of this novel IVIg preparation.</p>\",\"PeriodicalId\":9619,\"journal\":{\"name\":\"Case Reports in Dermatology\",\"volume\":null,\"pages\":null},\"PeriodicalIF\":0.9000,\"publicationDate\":\"2024-08-27\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11521433/pdf/\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Case Reports in Dermatology\",\"FirstCategoryId\":\"1085\",\"ListUrlMain\":\"https://doi.org/10.1159/000540519\",\"RegionNum\":0,\"RegionCategory\":null,\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"2024/1/1 0:00:00\",\"PubModel\":\"eCollection\",\"JCR\":\"Q4\",\"JCRName\":\"DERMATOLOGY\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Case Reports in Dermatology","FirstCategoryId":"1085","ListUrlMain":"https://doi.org/10.1159/000540519","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"2024/1/1 0:00:00","PubModel":"eCollection","JCR":"Q4","JCRName":"DERMATOLOGY","Score":null,"Total":0}
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.