A Case of Primary Systemic Amyloidosis Confirmed by Various Diagnostic Tools and Repeated Skin Biopsies / 대한피부과학회지

ABSTRACT

Amyloidosis is a group of disorders resulting from the extracellular deposition of amyloid fibrils in tissues and organs. Primary systemic amyloidosis may be myeloma-associated or idiopathic. It involves the kidney, heart, liver, peripheral nerves, autonomic nervous system and skin. We report a case of a 76 year-old woman with primary systemic amyloidosis who suffered from ecchymotic purpura on the periorbital, flexural area with hemorrhagic bulla, and macroglossia for two years. She showed typical symptoms of AL amyloidosis, and while primary systemic amyloidosis was suspected from electrophoresis results, no amyloid was found in the skin, tongue, and bone marrow. Upon her admission due to panperitonitis from diverticulitis, she was diagnosed with primary systemic amyloidosis after amyloid deposition was confirmed in the skin and colon biopsy. She had been treated with bortezomib, but she expired from methicillin-resistant Staphylococcus aureus septic shock.