title: 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.affiliation: Dermatology Department, University Hospital of Salamanca, Salamanca, Spain.
authors.forename: Javier
authors.initials: J
authors.lastname: Cañueto
authors.forename: Jorge
authors.initials: J
authors.lastname: Labrador
authors.forename: Concepción
authors.initials: C
authors.lastname: Román
authors.forename: Angel
authors.initials: A
authors.lastname: Santos-Briz
authors.forename: Teresa
authors.initials: T
authors.lastname: Contreras
authors.forename: Norma Carmen
authors.initials: NC
authors.lastname: Gutiérrez
authors.forename: Ramón
authors.initials: R
authors.lastname: García-Sanz
mesh_terms: D000328:Adult; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use*; D001897:Boronic Acids / Q000008:administration & dosage; D000069286:Bortezomib; D003907:Dexamethasone / Q000008:administration & dosage; D005260:Female; D006801:Humans; D011719:Pyrazines / Q000008:administration & dosage; D053718:Scleromyxedema / Q000188:drug therapy*
chemical_list: D001897:Boronic Acids; D011719:Pyrazines; D000069286:Bortezomib; D003907:Dexamethasone