Systemic amyloidosis. A report of 19 cases.

Systemic amyloidosis is a rare disorder and usually occurs in aged persons with a poor prognosis. Certain mucocutaneous symptoms and signs may act as important clues for diagnosis. Skin or tongue biopsy may also provide important information to establish a firm diagnosis. During the past 14-year period, we found 19 cases of systemic amyloidoses: 8 (42%) with primary systemic amyloidosis, 8 (42%) associated with multiple myeloma and 3 (16%) were secondary amyloidosis. Ages of the patients ranged from 52 to 71 years with a mean age of 62 years at diagnosis. Median survival was only 8 months. Mucocutaneous manifestations were found in both primary type and myeloma-associated amyloidosis. In addition to the existence of amyloid in tongue and rectum biopsies in 17 cases and abdominal fat aspiration in 1 case, biopsies from clinically normal-looking skin also showed positive results in 18 out of 19 cases.