文献简介

出版社:Springer-Verlag

作  者:Zdenek Adam, Marta Krejčí

编  号:10.1007/s00508-010-1361-x

关键字:IgA pemphigus, subcorneal pustular dermatosis, bortezomib, rituximab, monoclonal gammopathy.

年  份:2010   点击量:484

文献摘要

Summary. Monoclonal gammopathy-associated IgA pemphigus is a debilitating skin disorder with inconsistent response to treatment. A 61-year-old woman with IgA pemphigus and monoclonal gammopathy of unknown signifi cance had been treated unsuccessfully with cyclophosphamide/ dexamethasone and then with rituximab. When the monoclonal gammopathy progressed to multiple myeloma, the patient received treatment with cyclophosphamide/doxorubicin/dexamethasone but there was no clinical response. Second-line therapy with a thalidomide/cyclophosphamide/dexamethasone combination led to severe exacerbation of the skin disorder. However, therapy with a combination regimen that included bortezomib, cyclophosphamide and dexamethasone resulted in complete and durable remission of multiple myeloma and IgA pemphigus. Th is suggests that bortezomib-based therapy is useful for the treatment of the rare dermatologic disorder associated with IgA gammopathy.