Emily R. Gordon, Caroline Chen, Oluwaseyi Adeuyan, Brigit A. Lapolla, Megan H. Trager, Celine M. Schreidah, Lauren M. Fahmy, Larisa J. Geskin
<p>Monoclonal gammopathy of undetermined significance (MGUS) is a clonal plasma cell disorder characterized by monoclonal immunoglobulins or an abnormal ratio of free immunoglobulin light chains in blood or urine. Affecting 5% of adults aged 50 and older, MGUS is often asymptomatic and is typically incidentally diagnosed. In most cases, it requires only conservative observation and follow-up. However, MGUS may result in severe complications, such as significant cutaneous morbidity, which is not currently considered part of the diagnostic ‘requirement’ for multiple myeloma.<span><sup>1, 2</sup></span></p><p>Dermatologic manifestations linked to MGUS, which we named ‘gammopathic dermopathy’, include cutaneous light chain amyloidosis, POEMS syndrome, and scleromyxedema, among others.<span><sup>3-6</sup></span> Despite their existence, cutaneous MGUS manifestations remain poorly understood, leading to diagnostic delays and patient morbidity.<span><sup>7, 8</sup></span></p><p>While there are numerous multiple myeloma support groups, no official groups exist for those with MGUS. These patients struggle to find appropriate care and have difficulty obtaining effective medications because of the lack of diagnostic recognition, challenges with insurance coverage, and increased cost of medications. Thus, we organized the inaugural support group through Columbia Dermatology for patients with gammopathic dermopathy, titled ‘MGUS 4 Us’, to better understand the experiences of these patients.</p><p>Ten patients attended the meeting, sharing stories that highlighted common themes such as uncertainty and confusion about diagnosis, challenges in communicating diagnoses with families, difficulties in finding doctors with expertise, and struggles in obtaining effective treatment.</p><p>All 10 patients completed an optional survey with skin conditions reported including eczema, pyoderma gangrenosum, and scleromyxedema (Table 1). Notably, at least five patients experienced skin symptoms without an established diagnosis. Symptoms included itching (70%), pain (20%), rash (20%), and tightness (10%). Timelines varied, with one patient recently diagnosed with MGUS, five diagnosed 1–5 years ago, three diagnosed 5–10 years ago, and one diagnosed over 10 years ago. Two patients developed skin symptoms this year, three developed symptoms 1–5 years ago, two developed symptoms 5–10 years ago, two developed symptoms over 10 years ago, and one was unknown.</p><p>Of the 10 patients, six received treatment for skin symptoms, including topical steroids (4), multiple myeloma drugs (lenalidomide/ixazomib, 2), hydroxychloroquine (1), intravenous immunoglobulin (1), oral steroids (1), antibiotics (1), and dupilumab (1). While one patient reported feeling ‘a lot better’ after treatment, three felt ‘a little better’, two reported no change, and four did not receive treatment. The survey also revealed that most patients had dermatologists, oncologists, haematologists or rheumatologists invo
{"title":"Experiences of patients with cutaneous manifestations of monoclonal gammopathy of undetermined significance (MGUS): Insights from the first disease-specific support group","authors":"Emily R. Gordon, Caroline Chen, Oluwaseyi Adeuyan, Brigit A. Lapolla, Megan H. Trager, Celine M. Schreidah, Lauren M. Fahmy, Larisa J. Geskin","doi":"10.1002/jvc2.427","DOIUrl":"https://doi.org/10.1002/jvc2.427","url":null,"abstract":"<p>Monoclonal gammopathy of undetermined significance (MGUS) is a clonal plasma cell disorder characterized by monoclonal immunoglobulins or an abnormal ratio of free immunoglobulin light chains in blood or urine. Affecting 5% of adults aged 50 and older, MGUS is often asymptomatic and is typically incidentally diagnosed. In most cases, it requires only conservative observation and follow-up. However, MGUS may result in severe complications, such as significant cutaneous morbidity, which is not currently considered part of the diagnostic ‘requirement’ for multiple myeloma.<span><sup>1, 2</sup></span></p><p>Dermatologic manifestations linked to MGUS, which we named ‘gammopathic dermopathy’, include cutaneous light chain amyloidosis, POEMS syndrome, and scleromyxedema, among others.<span><sup>3-6</sup></span> Despite their existence, cutaneous MGUS manifestations remain poorly understood, leading to diagnostic delays and patient morbidity.<span><sup>7, 8</sup></span></p><p>While there are numerous multiple myeloma support groups, no official groups exist for those with MGUS. These patients struggle to find appropriate care and have difficulty obtaining effective medications because of the lack of diagnostic recognition, challenges with insurance coverage, and increased cost of medications. Thus, we organized the inaugural support group through Columbia Dermatology for patients with gammopathic dermopathy, titled ‘MGUS 4 Us’, to better understand the experiences of these patients.</p><p>Ten patients attended the meeting, sharing stories that highlighted common themes such as uncertainty and confusion about diagnosis, challenges in communicating diagnoses with families, difficulties in finding doctors with expertise, and struggles in obtaining effective treatment.</p><p>All 10 patients completed an optional survey with skin conditions reported including eczema, pyoderma gangrenosum, and scleromyxedema (Table 1). Notably, at least five patients experienced skin symptoms without an established diagnosis. Symptoms included itching (70%), pain (20%), rash (20%), and tightness (10%). Timelines varied, with one patient recently diagnosed with MGUS, five diagnosed 1–5 years ago, three diagnosed 5–10 years ago, and one diagnosed over 10 years ago. Two patients developed skin symptoms this year, three developed symptoms 1–5 years ago, two developed symptoms 5–10 years ago, two developed symptoms over 10 years ago, and one was unknown.</p><p>Of the 10 patients, six received treatment for skin symptoms, including topical steroids (4), multiple myeloma drugs (lenalidomide/ixazomib, 2), hydroxychloroquine (1), intravenous immunoglobulin (1), oral steroids (1), antibiotics (1), and dupilumab (1). While one patient reported feeling ‘a lot better’ after treatment, three felt ‘a little better’, two reported no change, and four did not receive treatment. The survey also revealed that most patients had dermatologists, oncologists, haematologists or rheumatologists invo","PeriodicalId":94325,"journal":{"name":"JEADV clinical practice","volume":"3 4","pages":"1294-1297"},"PeriodicalIF":0.0,"publicationDate":"2024-04-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1002/jvc2.427","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142123407","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}