The clinical manifestations of primary systemic amyloidosis with the involvement of a variety of organs, such as the kidney, heart, peripheral nervous system, liver, and so forth, are varied, complicated and nonspecific. The mucocutaneous manifestations, sometimes as the symptom in the early stages of disease, may provide an important clue for the diagnostic suspicion. Here we described a case of primary systemic amyloidosis with the history of Type 2 diabetes mellitus (T2DM) for nine years who had recurrent periorbital purpura in the initial stage, followed by nephrotic syndrome. The diagnosis was primary systemic amyloidosis finally determined by electron microscopy of renal pathology.
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