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Therapeutic Approach

Scleromyxedema is a rare and distinctive fibrosing mucinosis involving skin and other organs often associated with monoclonal gammopathy of undetermined significance (MGUS). Multidisciplinary care with oncology is essential. Treatments for scleromyxedema have not been adequately investigated with prospective or comparative clinical studies to establish reliable therapeutic guidelines. The majority of published literature on therapy is comprised of retrospective reports, which are inherently biased toward responsive cases and do not offer insight into relative efficacy of treatments. Further research is needed to delineate the most effective management strategy for these patients. Even with therapy, this disease is often progressive with substantial morbidity and mortality risk.1 IVIG is currently considered to be first-line therapy.1,2 Immunosuppression with glucocorticoids, lenolidamide, or thalidomide should be added for those with progressive or poorly responsive disease.1,3,4 Cytoreduction with melphalan,5-7 bortezomib + dexamethasone,8-10 or autologous stem cell transplant11,12 should be considered for those with severe or refractory disease in consultation with oncology.1 Interferon alpha-2b1,13,14 may be effective, but was recently discontinued and was not included in the treatment table. Extracorporeal photopheresis is well-tolerated and may be effective as adjunctive therapy for both systemic and cutaneous disease.4,6,15 Other treatments have demonstrated variable efficacy and may be useful in refractory cases. Combination therapy with multiple agents may be necessary to achieve an adequate response. Relapse and progression are common, so all patients should have regular short- and long-term follow-up, even if they initially respond well to therapy.1,5,16 See Table 54-1.

Table 54-1Scleromyxedema Treatment Table

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