Treatment of scleromyxedema with lenalidomide, bortezomib and dexamethasone: A case report and review of the literature

Abstract

This is an open access article under the terms of the Creative Commons Attribution-NonCommercial-NoDerivs License, which permits use and distribution in any medium, provided the original work is properly cited, the use is non-commercial and no modifications or adaptations are made.

Severe cutaneous scleromyxedema treated by lenalidomide

“…Other authors postulated that the M-protein acts as an antibody which directly stimulates fibroblast proliferation and mucin deposition. 3 Various treatments with inconsistent results have been described. 1,2 Systemic corticosteroids seem to be mainly a symptomatic treatment.…”

“…6 Lenalidomide stimulates NK-cell activity and increases IL-2 production, enhancing T-cell activity. 7 It has been used in various studies in the treatment of SM, in association with dexamethasone, IVIG or bortezomib, with daily dosages from 10 to 15 mg. 3,7,8 No treatment failures have been described. Duration of response has been variable, but no relapses were noted.…”

“…9 This is corroborated by the successful use of bortezomib and autologous stem cell transplant, which are well-established treatments of multiple myeloma, to treat SM. 3,9,10 However, clinical response of SM to lenalidomide and other treatments has not been consistently mirrored by decreased M-protein levels. 7,8,10 Isabel Illa et al showed that in vitro, fibroblasts could be activated by the serum of the patients with SM, but not with the purified monoclonal component alone.…”

Dermato-neuro Syndrome after COVID-19 Vaccination in a Patient with Scleromyxoedema Previously Controlled with Bortezomib and Intravenous Immunoglobulins

“…It is commonly used to treat relapsed/refractory multiple myeloma. Its use in SMX has been reported in 8 patients who differed in terms of clinical history, presentation and outcome, as well as treatment regimens (4)(5)(6)(7)(8)(9)(10)(11)(12)(13).…”

Cutaneous manifestations of monoclonal gammopathy

“…In context of dermato-neuro syndrome, the combination of IVIg and systemic corticosteroids are the treatment of choice according to European guidelines [ 115 ]. Lenalidomide [ 116 ] or bortezomib therapy [ 117 – 119 ] may achieve rapid improvement of cutaneous and systemic manifestations of scleromyxedema [ 120 ]. However, more studies addressing these issues are warranted.…”