January 2014
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1 Citation
International Journal of Case Reports and Images
Introduction: Primary systemic amyloidosis arises from clonal B cell disorder and may be associated with myeloma or lymphoma. Amyloid deposition can occur in any organ in the body as well as skin lesions. Both sexes are affected by this disease, with onset most commonly in the sixth decade or after. Case Report: A 26-year-old female was presented with gradually changing quality of voice, diffuse pigmentation all over body and lymphadenopathy in upper cervical group. Biopsy of a lymph node showed follicular hyperplasia with a few discrete large cells. Over a period of time, she developed purpuric rash over extremities and periorbital area, respiratory distress and heart failure. Echocardiography revealed cardiac amyloidosis and rectal biopsy with Congo red staining was positive for amyloidosis. Skin biopsy from hyperpigmented lesion of right forearm was also suggestive of amyloidosis and bone marrow examination showed plasma cell dyscrasia with a small M-band in serum protein electrophoresis. Finally, it was diagnosed a case of primary systemic amyloidosis.