Primary Esophageal Amyloidosis

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Abstract
Although primary amyloidosis has been recognized since the middle of the nineteenth century,1 little clinical interest was evidenced until Lubarsch2 reported 3 new cases in 1929. In 1935, Reimann, Kouchky, and Eklund3 presented the first case of primary amyloidosis diagnosed clinically. Since then, over 200 cases have been published. With the advent of these data, understanding of the protean manifestations of the disease has grown, and subsequently several classifications have been proposed. Probably the most widely accepted is that of Dahlin4: Primary Amyloidosis Systemic Tumefactive Amyloidosis complicating Myeloma Systemic Tumefactive Secondary Amyloidosis Systemic Tumefactive One year after this classification was published, a further subdivision of primary amyloidosis was recognized—familial amyloidosis.5 Since then, over 108 cases have been reported and indicate a definite clinical pattern (neuropathy, gastrointestinal and cardiovascular involvement, and ocular manifestations) with a simple dominant

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