PMID: 19947244Dec 2, 2009Paper

IgA pemphigus accompanying multiple myeloma has disappeared following the treatment with bortezomib (Velcade), cyclophosphamide and dexamethasone. Case study and literature review

Vnitr̆ní lékar̆ství
Zdenek AdamRoman Hajek

Abstract

IgA pemphigus, resembling subcorneal pustulous dermatosis, represents a rare complication of IgA type monoclonal gammopathy. The patient dates the onset of initial symptoms of vesicular-bullous disease to 1990. She was first examined at our clinic in 2001 with the following conclusion "type IgA monoclonal gammopathy of unknown significance". The first immunosuppressive treatment of vesicular-bullous disorder was administered in 2003 (dexamethasone 20 mg on days 1-4 and 15-18 in monthly cycles + daily cyclophosphamide 50 mg). Cyclophosphamide was administered for 6 months in total and dexamethasone for further 3 months. During the treatment, intensity of the skin disorder ameliorated and monoclonal IgA levels decreased to non-detectable levels. Nevertheless, skin symptoms recurred immediately after dexamethasone treatment in its original intensity was terminated, even though the concentration of monoclonal immunoglobulin IgA remained below the sensitivity of quantitative detection for further 6 months (positive immunofixation only). Six rituximab 600 mg infusions were administered in a weekly interval after stopping cyclophosphamide and dexamethasone to prevent early recurrence of skin symptoms but this treatment was without any...Continue Reading

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