Erythema elevatum diutinum (EED) is a chronic cutaneous disease characterized by nodules and plaques in an acral and symmetrical location. Histologically, the earliest findings are a vasculitis in the dermis associated with an inflammatory-cell infiltrate and a tinctorial change in the vessel wall and adjacent perivascular fibers. The histologic findings are thought to be highly characteristic. Extracellular cholesterosis (EC) most likely represents a variant in which lipids are secondarily deposited between the collagen bundles. The earlier division of EED into the Hutchinson and Bury types is no longer thought to be warranted. A patient is presented with clinical and histological features of EED; an elevation of several amino acids in the urine with essentially normal plasma levels was revealed. Elevations of IgG and IgA, and a mild elevation in leukocyte alkaline phosphatase were present.