[Difficulties in the diagnosis of hereditary familial amyloidosis]

A 24-year-old woman had fever with skin rash, marked hepatolienal syndrome from the age of 1.5 years followed in later life by involvement of central nervous system and kidneys. Morphologically, there was accumulation of amyloid FAP in hepatic and rectal tissues. Family history of amyloidosis and the above evidence confirmed hereditary nature of the disease.