Severe cutaneous scleromyxedema treated by lenalidomide

Autor: Théo Brochet, Ali Dadban, Quentin Beauvillain, Clemence Boucher, Fanny Dessirier, Christophe Attencourt, Catherine Lok, Pierre Morel, Aurélie Halle, Guillaume Chaby
Jazyk: angličtina
Rok vydání: 2023
Předmět:
Zdroj: JEADV Clinical Practice, Vol 2, Iss 3, Pp 608-611 (2023)
Druh dokumentu: article
ISSN: 2768-6566
DOI: 10.1002/jvc2.159
Popis: Abstract Scleromyxedema is a rare clinical variant of primary cutaneous mucinosis. It is defined by a generalized papular and sclerodermoid eruption associated with monoclonal gammopathy. No specific definitive treatment is known and those tried have inconsistent results. The first‐line treatment is intravenous immunoglobulin infusions with variable response and frequent relapse. We describe, here, a patient with scleromyxedema with lambda monoclonal gammopathy treated effectively with low‐dose lenalidomide and dexamethasone. There was no clinical or biological relapse after 3 years of follow‐up. In our opinion, agents targeting the abnormal plasma cell clone like lenalidomide are the most promising in the treatment of scleromyxedema.
Databáze: Directory of Open Access Journals
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