An 87-year-old woman presented for evaluation of progressive, nonpruritic, waxy papules and atraumatic ecchymoses in a periorbital ( raccoon eyes, Panel A) and perioral distribution. She was otherwise asymptomatic. Examination of a biopsy specimen of a representative lesion revealed a nodular deposition of a hyalinized, amorphous material in the superficial dermis. Positive Congo red staining and apple-green birefringence under polarized light (Panel B) confirmed the presence of amyloid fibrils. Serum and urine electrophoresis with immunofixation revealed monoclonal kappa light-chain proteins. Levels of hemoglobin and serum calcium were unremarkable, as were the results of a radiographic skeletal survey. Renal function was stable, with only a mildly reduced glomerular filtration rate. Biopsy samples of bone marrow revealed that 22% of marrow cellularity consisted of plasma cells that stained predominantly for kappa light-chain proteins. A diagnosis of kappa light-chain multiple myeloma with systemic amyloidosis was made. After initial treatment with melphalan and prednisone, the patient’s condition stabilized, and at 2 years of follow-up she continued treatment with bortezomib alone.
W. Harris Green, M.D.
Robert Hill Schosser, M.D.
East Carolina University, Greenville, NC