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Wiener klinische Wochenschrift

, Volume 122, Issue 9–10, pp 311–314 | Cite as

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

  • Zdenek AdamEmail author
  • Marta Krejčí
  • Luděk Pour
  • Josef Feit
  • Tomáš Büchler
  • Roman Hájek
Case report

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 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.

Keywords

IgA pemphigus Subcorneal pustular dermatosis Bortezomib Rituximab Monoclonal gammopathy 

Komplette Remission von multiplem Myelom und IgA Pemphigus nach Kombinationstherapie mit Bortezomib, Cyclophosphamid und Dexamethason

Zusammenfassung

Der mit einer monoklonalen Gammopathie assoziierte IgA Pemphigus ist eine sehr belastende Hauterkrankung mit unterschiedlichem Ansprechen auf Therapie. Eine 61-jährige Frau mit einem IgA Pemphigus und einer monoklonalen Gammopathie "of unknown significance" wurde ohne Erfolg mit Cyclophosphamid/Dexamethason, gefolgt von Rituximab behandelt. Als die monoklonale Gammopathie in ein multiples Myelom überging, erhielt die Patientin eine Therapie mit Cyclophosphamid/Doxorubicin/Dexamethason – allerdings ohne Erfolg. Eine second-line Therapie mit der Kombination Thalidomid/Cyclophosphamid/Dexamethason führte zu einer Exazerbation der Hauterkrankung. Schließlich führte die Kombinationstherapie mit Bortezomib, Cyclophosphamid und Dexamethason zu einer kompletten und dauerhaften Remission sowohl des multplen Myeloms als auch des IgA Pemphigus. Unsere Erfahrung lässt vermuten, dass eine Bortezomib-basierte Therapie in der Behandlung der seltenen, mit einer IgA Gammopathie assoziierten Hauterkrankung wirkungsvoll ist.

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Copyright information

© Springer-Verlag 2010

Authors and Affiliations

  • Zdenek Adam
    • 1
    Email author
  • Marta Krejčí
    • 1
  • Luděk Pour
    • 1
  • Josef Feit
    • 2
  • Tomáš Büchler
    • 3
  • Roman Hájek
    • 1
  1. 1.Department of Internal Medicine – Hematooncology, Faculty of Medicine and University HospitalMasaryk UniversityBrnoCzech Republic
  2. 2.Institute of Pathology, Faculty of Medicine and University HospitalMasaryk UniversityBrnoCzech Republic
  3. 3.Department of Oncology, Thomayer University Hospital and 1st Faculty of MedicineCharles UniversityPragueCzech Republic

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