40528 Administration of bortezomib in patients with refractory scleromyxedema: A case report

Document Type

Conference Proceeding

Publication Date

9-19-2023

Publication Title

J Am Acad Dermatol

Abstract

Background: We present a case of refractory scleromyxedema, a rare cutaneous mucinosis, treated with bortezomib and dexamethasone. To our knowledge, this case documents the longest duration of remission to date. Case: A 58-year-old African American female presented with a one year history of scattered monomorphic erythematous cobblestone papules/plaques on bilateral legs and extensor arms. Workup was significant for monoclonal IgG kappa protein. Skin and bone marrow biopsy revealed mucin deposition and plasma cell myeloma, respectively. Initially, she responded to intravenous IVIG from 2014-2016, but was discontinued due to debilitating migraines. Approximately 60- 70% resolution of plaques was achieved with subcutaneous immunoglobulin, plaquenil, and prednisone. However, she eventually relapsed with increased thickened pruritic facial plaques. Dermatology recommended bortezomib and dexamethasone in 2019. She received three cycles but discontinued treatment due to bilateral leg neuropathy. Despite this, bortezomib and dexamethasone has resulted in a 3-year remission. Discussion: Untreated scleromyxedema results in diffuse pruritic papules/plaques, congestive heart failure, restrictive lung disease, and encephalopathy. Selection among treatment options remains difficult due to incomplete understanding and lack of randomized controlled trials (1). High-dose immunoglobulins are considered the treatment of choice, followed by thalidomide and systemic glucocorticosteroids (2). This case provides support of bortezomib and dexamethasone for refractory scleromyxedema. Our patient has remained in remission for three years, the longest documented to date. Notably, our case achieved remission with fewer cycles compared to other cases, with prior studies citing a minimum of four cycles and a maximum of eight cycles (1,3).

Volume

89

Issue

3

First Page

AB117

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