A 75-year-old man with a past medical history of carpal tunnel syndrome and chronic dysphonia presented with a 2-year history of swelling in his hands. Physical examination revealed marked enlargement of the soft tissues in both palms and finger pads, with a translucent and edematous appearance that produced pitting on palpation (Fig. 1), as well as macroglossia and eyelid erythema. The patient reported asthenia and weight loss.
A skin biopsy of the left palm identified infiltration by Congo red-positive fibrillar material showing green birefringence under polarized light, consistent with amyloidosis. Additional studies revealed elevated light chains, proteinuria, amyloid infiltration of the myocardium on positron emission tomography, and bone marrow infiltration by abnormal plasma cells. These findings were consistent with a diagnosis of primary systemic amyloidosis.
The image illustrates an uncommon presentation of systemic amyloidosis, which more typically manifests as periorbital ecchymosis, petechiae, purpura, nodules or plaques in various regions of the cephalic pole, macroglossia, and translucent lesions in the anogenital area.
