ArticleJournal of the European Academy of Dermatology and Venereology : JEADV2025
Plasma cell-directed therapies induce profound clinical and durable responses in patients with severe or relapsed/refractory scleromyxedema.
Article in Journal of the European Academy of Dermatology and Venereology : JEADV, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 5 papers.
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Who cites it
5 citing papers in PubMed.
- Dupilumab improves itch, but not fibrosis, in a patient with scleromyxedema: A case report.SAGE open medical case reports · 2026Article
- Sclerosing diseases of the skin.Journal der Deutschen Dermatologischen Gesellschaft = Journal of the German Society of Dermatology : JDDG · 2025Review
- Monoclonal Gammopathies of Clinical Significance-Scleromyxedema: A Case Report and Literature Review.Clinical case reports · 2025Article
- Plasma cell-directed therapies induce profound clinical and durable responses in patients with severe or relapsed/refractory scleromyxedema.Journal of the European Academy of Dermatology and Venereology : JEADV · 2025Article
- Clonal disorders of clinical significance. A concept with important therapeutic implications.Journal of the European Academy of Dermatology and Venereology : JEADV · 2025Article
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Abstract
backgroundScleromyxedema (SM) is a rare skin disorder related to monoclonal gammopathy. High dose intravenous immunoglobulins (HDIVIg) are usually used as a frontline therapy with initial efficacy. However, some patients evolve with relapse, refractory state or severe extra-cutaneous complications such as dermato-neuro syndrome (DNS) or cardiac involvement. The objective of the study is to evaluate the use of anti-plasma cell treatment in these patients in order to obtain a deep and durable dermatological and haematological response.
methodsWe report here eight patients treated with HDIVIg together with anti-plasma cell therapy including: lenalidomide and dexamethasone (n = 5); bortezomib, cyclophosphamide and dexamethasone (n = 1); daratumumab, lenalidomide and dexamethasone (n = 2).
resultsCombination of HDIVIg with a treatment targeting the monoclonal component led to a high level of haematological remission and drastically improved skin response with an acceptable safety profile in all patients. Moreover, HDIVIg was reduced and stopped in 4 of the 7 patients who achieved complete remission.
conclusionsThe association of lenalidomide and dexamethasone with HDIVIg could improve the treatment of relapsed or severe SM.
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