Amyloidosis is a group disease which progresses with extracellular accumulation of fibrillary protein in beta conformation structure. It is categorized as localized amyloidosis when storage is limited to one organ (i.e. on skin) or systemic (generalized) amyloidosis when it occurs with several organ involvement. In this article, a primary systemic amyloidosis case diagnosed with skin lesions is presented with the purpose of reminding primary systemic amyloidosis in differential diagnosis of periorbital ecchymosis and reviewing the findings of primary systemic amyloidosis which is a rarely seen disease in dermatology practice.
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