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Discussion

Erythma elevatum diutinum (EED) is a rare chronic leukocytoclastic vasculitis of unknown etiology1, 2 characterized by violaceous, red to purple papules, plaques or nodules which tend to be distributed symmetrically on the extensor surfaces of the extremities. 1, 3 Our case was atypical in that warty lesions developed on the palms and soles. The cause of EED is unknown but several studies have suggested immune complex mechanisms. The disease occurs in association with a variety of conditions including autoimmunity, infectious diseases, for example, streptococci, HIV, 4 and hematological abnormalities including myelodysplasia, 5 multiple myeloma, 6 lymphoma, 3 paraproteinemias, 7 and certain malignancies such as glioma, squamous cell carcinoma and breast carcinoma.

As mentioned previously, a number of cases of EED occurring in patients with paraproteinemias have been identified. IgA is the most common isotype with over 20 reported cases. 8 The temporal relationship of EED and paraproteinemia has not been established and EED severity does not seem to be dependent on total paraprotein levels, regardless, IgA paraprotein level has been suggested as a marker for EED and immunoelectrophoresis screening for monoclonal gammapathies has been recommended in all patients with EED. 9 Histopathologically, acute lesions display a neutrophilic, perivascular infiltrate with dermal fibrin deposits, endothelial expansion, and leukocytoclasis. Vessel occlusion may occur and rarely the epithelium may show secondary ischemic necrosis. Mature lesions show dermal fibrosis and vasculature prominence with persistent …