Scleromyxedema Managed With High-Dose Intravenous Immunoglobulin and Bortezomib-Dexamethasone: A Case Report

Scleromyxedema is a rare, chronic cutaneous mucinosis marked by widespread waxy papules and potential extracutaneous involvement. This case report discusses the management of a 48-year-old female diagnosed with scleromyxedema, who initially partially responded to high-dose intravenous immunoglobulin (HDIVIG) therapy. After partial relapse, she was induced with bortezomib, a proteasome inhibitor, and dexamethasone, achieving significant clinical improvement. Long-term maintenance with IVIG was utilized to prevent recurrence of symptoms. This case highlights the effectiveness of both HDIVIG and bortezomib-dexamethasone dual therapy as viable treatment options for scleromyxedema, emphasizing the importance of maintenance therapy to prevent relapse.