Rkp. Chow et al., ERYTHEMA-ELEVATUM-DIUTINUM ASSOCIATED WITH IGA PARAPROTEINEMIA SUCCESSFULLY CONTROLLED WITH INTERMITTENT PLASMA-EXCHANGE, Archives of dermatology, 132(11), 1996, pp. 1360-1364
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.