Development of amyloidosis in Behçet's syndrome with isolated mucocutaneous involvement.

Development of secondary amyloidosis is an infrequent complication in patients with Behçet's syndrome (BS). It has been reported that multiple systemic involvement, long disease duration, and male gender are major clinical factors accompanying the development of amyloidosis in BS. We report a case of secondary amyloidosis in a patient diagnosed as having BS with a positive pathergy test 9 years previously and who had isolated mucocutaneous involvement. Regular use of colchicine since the diagnosis and somewhat mild progress of the disease could not prevent the development of secondary amyloidosis in this patient. He is alive and receives hemodialysis regularly.