Dermatol. praxi. 2010;4(4):221-224

IgA pemphigus and monoclonal gammopathy, is there a connection ?

prof.MUDr.Zdeněk Adam, CSc.¹, doc.MUDr.Josef Feit, CSc.², doc.MUDr.Marta Krejčí, Ph.D.¹, MUDr.Luděk Pour, Ph.D.¹, doc.MUDr.Vladimír Vašků, CSc.³, MUDr.Zdeňka Čermáková⁴, prof.MUDr.Roman Hájek, CSc.¹, prof.MUDr.Jiří Mayer, CSc.¹

¹ Interní hematoonkologická klinika LF MU a FN Brno

² Ústav patologie LF MU a FN Brno

³ Dermatologická klinika LF MU a FN u sv. Anny Brno

⁴ Oddělení klinické biochemie Fakultní nemocnice Brno

Monoclonal gammopathy-associated IgA pemphigus is a debilitating skin disorder with inconsistent response to treatment. A 61-year

old female patient 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 thalidomide/cyclophosphamide/dexamethasone combination led to severe exacerbation of the skin disorder. However, therapy with

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 and that monoclonal immunoglobulin of IgA class was the cause of IgA pemphigus.

Keywords: IgA pemphigus, subcorneal pustular dermatosis, bortezomib, rituximab, monoclonal gammopathy

Published: December 10, 2010  Show citation