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. 2025 May;39(5):1011-1016.
doi: 10.1111/jdv.20257. Epub 2024 Jul 31.

Plasma cell-directed therapies induce profound clinical and durable responses in patients with severe or relapsed/refractory scleromyxedema

F Theves  1 , C Lahuna  1 , T Mahévas  2 , S Harel  1 , B Royer  1 , V Lemiale  3 , A Brignier  4 , N Parquet  4 , M Jachiet  2 , J-D Bouaziz  2 , D Elessa  1 , B Arnulf  1 , A Talbot  1
Affiliations

Plasma cell-directed therapies induce profound clinical and durable responses in patients with severe or relapsed/refractory scleromyxedema

F Theves et al. J Eur Acad Dermatol Venereol. 2025 May.

Abstract

Background: Scleromyxedema (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.

Methods: We 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).

Results: Combination 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.

Conclusions: The association of lenalidomide and dexamethasone with HDIVIg could improve the treatment of relapsed or severe SM.

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Conflict of interest statement

Pr Arnulf has competing interests with BMS celgene, Dr Talbot has competing interests with BMS Celgene, Dr Brignier has competing interests with BMS Celgene, Janssen and Octapharma, Dr Theves has competing interests with Sanofi and Janssen. The authors Dr Lahuna, Dr Mahevas, Dr Harel, Dr Royer, Dr Lemiale, Dr Parquet, Dr Jachet, Pr Bouaziz and Dr Elessa have no competing interests in this article. No support found this work.

Figures

FIGURE 1
FIGURE 1
Picture of patients before and after HDIVIg and lenalidomide.
See this image and copyright information in PMC

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