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

TL;DR

A 48-year-old woman with scleromyxedema showed significant improvement using a combination of high-dose immunoglobulin and bortezomib-dexamethasone, with maintenance therapy preventing relapse.

Contribution

This case report presents a successful treatment approach combining HDIVIG and bortezomib–dexamethasone for scleromyxedema.

Findings

Partial response to high-dose intravenous immunoglobulin (HDIVIG) was observed.

Significant clinical improvement occurred with bortezomib and dexamethasone.

Long-term maintenance with IVIG prevented symptom recurrence.

Abstract

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.

Figures