ERYTHEMA-ELEVATUM-DIUTINUM ASSOCIATED WITH IGA PARAPROTEINEMIA SUCCESSFULLY CONTROLLED WITH INTERMITTENT PLASMA-EXCHANGE

Background: We review the literature and report a case of refractory e rythema elevatum diutinum associated with IgA paraproteinemia that was successfully controlled with intermittent plasma exchange (PLEX). Obs ervations: Typical lesions of erythema elevatum diutinum developed in a 72-year-old patient with IgA paraproteinemia; the condition predicta bly flared whenever IgA levels reached a threshold of 8 g/L. After 8 y ears of unsuccessful treatment with various agents, we instituted a tr ial of PLEX during an acute flare. Following 6 exchanges over a period of 2 weeks, the IgA level decreased from 8 to 2 g/L and the skin lesi ons cleared. Three weeks later, new skin lesions developed and the IgA level had rebounded from 2 to just over 8 g/L. A second course of PLE X was administered, with excellent results, and a 3-month course of or al chlorambucil (2 mg/d) was initiated. The patient's condition remain ed in clinical remission for 10 months. Over the ensuing 9 years, she suffered 11 further flares, each of which was associated with IgA leve ls of 8 to 10 g/L and each responding dramatically to 3 to 5 PLEXs fol lowed by a consolidative dose of intravenous cyclophosphamide (250-500 mg). Conclusion: We believe that PLEX may have an important role in t he management of severe erythema elevatum diutinum associated with mon oclonal paraproteinemia refractory to other therapy.