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Primary systemic amyloidosis

Secondary to abnormal protein deposition in the skin, which in turn is often related to multiple myeloma, primary systemic amyloidosis is manifested in the skin by macroglossia, hemorrhagic papules involving the central face and eyelids, marked tendency of the skin to develop petechia and, if infiltration is extensive, a patchy alopecia. The petechia can develop seconds after a scratch or other minor trauma. The facial plaques are typically described as waxy or translucent, unless they are purpuric.
Systemic involvement includes, most significantly, renal involvement, which can lead to renal failure.

Treatment:

  • Treatment of myeloma with melphalan can markedly improve primary systemic amyloidosis.
primary systemic amyloidosis


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