Extended survival of patients with primary systemic amyloidosis.

Systemic amyloidosis can produce a wide variety of clinical manifestations, including characteristic cutaneous findings. Large series of patients with primary systemic amyloidosis have shown that systemic amyloidosis, with or without associated myeloma, has a median survival of no more than twenty-four months. We present a case of systemic amyloidosis that has been present in a woman for eighteen years, as manifested by periorbital purpura and an immunoglobulin G kappa light chain paraproteinemia. She was otherwise healthy; results of bone marrow examination showed no overt myeloma. We speculate that kappa light chain paraproteinemia could prove to be a marker for a more benign type of systemic amyloidosis.