IgA pemphigus associated with monoclonal gammopathy completely resolved after achievement of complete remission of multiple myeloma with bortezomib, cyclophosphamide and dexamethasone regimen

Wien Klin Wochenschr. 2010 May;122(9-10):311-4. doi: 10.1007/s00508-010-1361-x.

Abstract

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 significance 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. This suggests that bortezomib-based therapy is useful for the treatment of the rare dermatologic disorder associated with IgA gammopathy.

Publication types

  • Case Reports
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Antineoplastic Combined Chemotherapy Protocols / administration & dosage*
  • Boronic Acids / administration & dosage
  • Bortezomib
  • Cyclophosphamide / administration & dosage
  • Dexamethasone / administration & dosage
  • Female
  • Humans
  • IgA Deficiency / complications
  • IgA Deficiency / prevention & control*
  • Middle Aged
  • Multiple Myeloma / complications
  • Multiple Myeloma / drug therapy*
  • Paraproteinemias / complications
  • Paraproteinemias / prevention & control*
  • Pemphigus / complications
  • Pemphigus / prevention & control*
  • Pyrazines / administration & dosage
  • Remission Induction

Substances

  • Boronic Acids
  • Pyrazines
  • Bortezomib
  • Dexamethasone
  • Cyclophosphamide