We describe a female patient with primary Sjögren's syndrome (SS) associated with a subcutaneous tumor formed by amyloid surrounded by mature IgG-kappa plasma cells, in the absence of lymphoproliferation or amyloid deposit in other organs. This case represents an unusual presentation of localized amyloidosis, without systemic involvement. Together with other reported cases this suggests that only immunocytic and not reactive systemic amyloidosis develops in chronic SS patients.
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