Plasma cell-directed therapies in monoclonal gammopathy-associated scleromyxedema

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TitrePlasma cell-directed therapies in monoclonal gammopathy-associated scleromyxedema
Type de publicationJournal Article
Year of Publication2020
AuteursMahevas T, Arnulf B, Bouaziz J-D, Livideanu CBulai, Osio A, Servy A, Cribier B, Sassolas B, Jachiet M, Michel L, Aucouturier P, Lipsker D, Frances C, Sbidian E, Rybojad M, Descamps V, D'Incan M, Humbert P, Beylot-Barry M, Passeron T, de Moreuil C, Taha RY, Hermine O, Dupuy A, Barbarot S, Debarbieux S, Carpentier O, Brault F, Schmutz J-L, Thomas-Beaulieu D, Modiano P, Zarnitsky C, Lifermann F, Baubion E, Limal N, Le Bras F, Le Moigne M, Tauber M, Talbot A, Prud'homme R, Peltier S, De Masson A, Battistella M, Bagot M, Mekinian A, Fain O, Dermatolo EMaladies S, Interne SNatl Med, Disorders FNetwork Dy, Immuno GEtude Derm
JournalBLOOD
Volume135
Pagination1101-1110
Date PublishedAPR 2
Type of ArticleArticle
ISSN0006-4971
Résumé

{Scleromyxedema is a rare skin and systemic mucinosis that is usually associated with monoclonal gammopathy (MG). In this French multicenter retrospective study of 33 patients, we investigated the clinical and therapeutic features of MG-associated scleromyxedema. Skin molecular signatures were analyzed using a transcriptomic approach. Skin symptoms included papular eruptions (100%), sclerodermoid features (91%), and leonine facies (39%). MG involved an immunoglobulin G isotype in all patients, with a predominant K light chain (73%). Associated hematologic malignancies were diagnosed in 4 of 33 patients (12%) (smoldering myeloma
DOI10.1182/blood.2019002300