Localized immunoglobulin light chain amyloid of the conjunctiva confirmed by mass spectrometry without evidence of systemic disease

Current guidelines recommend a thorough systemic workup in patients with newly diagnosed immunoglobulin amyloid light chain (AL) amyloidosis because of a close association with an underlying plasma cell proliferative disorder. Rarely, however, AL amyloidosis may present as a localized, benign disease without systemic involvement. Few previous reports have documented localized AL amyloid of the conjunctiva, and all these used immunohistochemistry for amyloid typing, which can be inaccurate. The authors identified 2 cases of AL amyloidosis confirmed by mass spectrometry localized to the conjunctiva and without evidence of systemic disease as determined by thorough systemic workup. Despite the very sensitive diagnostic technique of mass spectrometry, AL amyloidosis of the conjunctiva may not have an association with systemic plasma cell proliferative disorder. It is important to recognize conjunctiva as a site where localized amyloidosis can occur, even with the AL subtypes, as this entity usually exhibits a benign course and may be managed conservatively.