Patients with primary systemic or myeloma-associated amyloidosis have multiple nonspecific constitutional symptoms, such as fatigue, weight loss and edema. The classic presentation of macroglossia, carpal tunnel syndrome, hepatomegaly and cutaneous lesions is uncommon. Other systemic findings can include CHF or arrhythmias due to cardiac infiltration, splenomegaly, and peripheral and autonomic neuropathies. Purpuric lesions are classically found at the eyelids, or flexural areas such as the neck and axillae. Again, these occur most frequently in flexural areas.
Diagnosis is based on characteristic skin biopsy findings.
Once the diagnosis of systemic amyloidosis is made, an evaluation should be undertaken to screen for myeloma. This may include serum and urine protein electrophoresis, as well as bone marrow biopsy and radiographic screening. Chemotherapy, with or without bone marrow transplantation is can be used in the treatment of systemic amyloidosis. Touart DM, Sau P. Cutaneous deposition diseases. Amyloid and amyloidosis.