Erythema Elevatum Diutinum Associated With IgA Paraproteinemia Successfully Controlled With Intermittent Plasma Exchange

Abstract

BACKGROUND: We review the literature and report a case of refractory erythema elevatum diutinum associated with IgA paraproteinemia that was successfully controlled with intermittent plasma exchange (PLEX). OBSERVATIONS: Typical lesions of erythema elevatum diutinum developed in a 72-year-old patient with IgA paraproteinemia; the condition predictably flared whenever IgA levels reached a threshold of 8 g/L. After 8 years of unsuccessful treatment with various agents, we instituted a trial 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 lesions 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 PLEX was administered, with excellent results, and a 3-month course of oral chlorambucil (2 mg/d) was initiated. The patient's condition remained in clinical remission for 10 months. Over the ensuing 9 years, she suffered 11 further flares, each of which was associated with IgA levels of 8 to 10 g/L and each responding dramatically to 3 to 5 PLEXs followed by a consolidative dose of intravenous cyclophosphamide (250-500 mg). CONCLUSION: We believe that PLEX may have an important role in the management of severe erythema elevatum diutinum associated with monoclonal paraproteinemia refractory to other therapy.