title: Primary sjögren syndrome manifested as localized cutaneous nodular amyloidosis.
abstract: 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. Sjögren 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 Sjögren syndrome is considerable. Herein, we report a 78-year-old woman with LCNA, who was further surveyed and diagnosed with Sjögren syndrome. In light of the significant relation between these 2 diseases, further examination for coexistence of Sjögren syndrome in addition to systemic amyloidosis is well warranted. Prompt identification of an underlying Sjögren syndrome in LCNA with polyclonal immunoglobulin amyloid may have important therapeutic consequences.
authors.affiliation: Department of Dermatology, School of Medicine, Chung-Shan Medical University Hospital, Taichung, Taiwan.
authors.forename: Shiow-Jiuan
authors.initials: SJ
authors.lastname: Wey
authors.forename: Yi-Ming
authors.initials: YM
authors.lastname: Chen
authors.forename: Po-Ju
authors.initials: PJ
authors.lastname: Lai
authors.forename: Der-Yuan
authors.initials: DY
authors.lastname: Chen
mesh_terms: D000368:Aged; D000686:Amyloidosis / Q000175:diagnosis* / Q000188:drug therapy / Q000276:immunology; D018501:Antirheumatic Agents / Q000008:administration & dosage; D001379:Azathioprine / Q000008:administration & dosage; D005260:Female; D006801:Humans; D006886:Hydroxychloroquine / Q000008:administration & dosage; D007150:Immunohistochemistry; D011241:Prednisone / Q000008:administration & dosage; D012859:Sjogren's Syndrome / Q000175:diagnosis* / Q000188:drug therapy / Q000276:immunology; D012871:Skin Diseases / Q000175:diagnosis* / Q000188:drug therapy / Q000276:immunology
chemical_list: D018501:Antirheumatic Agents; D006886:Hydroxychloroquine; D001379:Azathioprine; D011241:Prednisone