Primary Sjogren Syndrome Manifested as Localized Cutaneous Nodular Amyloidosis

Localized cutaneous nodular amyloidosis (LCNA) is the rarest type of cutaneous amyloidosis. Typically presenting as waxy nodules on the lower extremities, it demonstrates localized deposition of AL-type amyloid in immunohistologic study and is often associated with focal plasma cell proliferation. Sjogren syndrome, an autoimmune lymphoproliferative disorder, is characterized by keratoconjunctivitis sicca and xerostomia with lymphocytic infiltration of exocrine glands. As shown in case reports, the association of LCNA with Sjogren syndrome is considerable. Herein, we report a 78-year-old woman with LCNA, who was further surveyed and diagnosed with Sjogren syndrome. In light of the significant relation between these 2 diseases, further examination for coexistence of Sjogren syndrome in addition to systemic amyloidosis is well warranted. Prompt identification of an underlying Sjogren syndrome in LCNA with polyclonal immunoglobulin amyloid may have important therapeutic consequences.