The combination of bortezomib and dexamethasone is an efficient therapy for relapsed/refractory scleromyxedema: a rare disease with new clinical insights.

Abstract

Scleromyxedema (SM) is a rare primary cutaneous inflammatory mucinosis characterised by papular mucinosis, monoclonal gammopathy and extracutaneous involvement. Most therapeutic options have failed in SM but high-dose therapy followed by autologous peripheral blood stem cell transplantation (APBSCT) appears to be highly effective, although SM normally relapses. We report the case of a 29-yr-old patient with severe SM who achieved stringent complete response with Bortezomib plus Dexamethasone after an early relapse subsequent to a high-dose melphalan regimen followed APBSCT. It is of particular note that dermatological lesions responded to both therapies before M-component modifications, suggesting that SM is independent of M-component characteristics. However, treatment should be directed towards the underlying plasma cell malignancy with typical anti-myeloma agents.

• Authors: Javier Cañueto, Jorge Labrador, Concepción Román, Angel Santos-Briz, Teresa Contreras, Norma Carmen Gutiérrez, Ramón García-Sanz
• Journal: European journal of haematology (Eur J Haematol) Vol. 88 Issue 5 Pg. 450-4 (May 2012) ISSN: 1600-0609 [Electronic] England
• PMID: 22404151 (Publication Type: Case Reports, Journal Article, Research Support, Non-U.S. Gov't)
• Copyright: © 2012 John Wiley & Sons A/S.

Chemical References

• Boronic Acids
• Pyrazines
• Bortezomib
• Dexamethasone

Topics

• Adult
• Antineoplastic Combined Chemotherapy Protocols (therapeutic use)
• Boronic Acids (administration & dosage)
• Bortezomib
• Dexamethasone (administration & dosage)
• Female
• Humans
• Pyrazines (administration & dosage)
• Scleromyxedema (drug therapy)