title: Sjögren's syndrome and localized nodular cutaneous amyloidosis: coincidence or a distinct clinical entity?
abstract: OBJECTIVE
  To report 8 patients with Sjögren's syndrome (SS) and localized nodular cutaneous amyloidosis and to examine serologic and immunohistologic findings that may link the 2 diseases.
  
  
  METHODS
  The databases for 3 amyloidosis centers were searched for patients with localized nodular cutaneous amyloidosis and SS. Eight patients with this combination were identified, and clinical, serologic, and histologic parameters were retrospectively evaluated.
  
  
  RESULTS
  Among the 8 patients with a clinical diagnosis of SS, 6 fulfilled the American-European Consensus Group criteria for SS. All of the patients were women in whom SS had been diagnosed at a median age of 47 years (range 30-61 years) and amyloid had been diagnosed at a median age of 60 years (range 42-79 years). The presence of the immunoglobulin light chain type of amyloid (AL amyloid) was confirmed in 4 patients. In 3 of these 4 patients as well as 2 other patients, a light chain-restricted plasma cell population was observed near the amyloid deposits. Progression to systemic amyloidosis was not observed in any patient during a median followup of 3.5 years.
  
  
  CONCLUSIONS
  SS should be considered in patients with cutaneous amyloidosis. The combination of cutaneous amyloidosis and SS appears to be a distinct disease entity reflecting a particular and benign part of the polymorphic spectrum of lymphoproliferative diseases related to SS.
authors.affiliation: University of Groningen, Groningen, The Netherlands.
authors.forename: Jiska M
authors.initials: JM
authors.lastname: Meijer
authors.forename: Stefan O
authors.initials: SO
authors.lastname: Schonland
authors.forename: Giovanni
authors.initials: G
authors.lastname: Palladini
authors.forename: Giampaolo
authors.initials: G
authors.lastname: Merlini
authors.forename: Ute
authors.initials: U
authors.lastname: Hegenbart
authors.forename: Olga
authors.initials: O
authors.lastname: Ciocca
authors.forename: Vittorio
authors.initials: V
authors.lastname: Perfetti
authors.forename: Martha K
authors.initials: MK
authors.lastname: Leijsma
authors.forename: Hendrika
authors.initials: H
authors.lastname: Bootsma
authors.forename: Bouke P C
authors.initials: BP
authors.lastname: Hazenberg
mesh_terms: D000328:Adult; D000368:Aged; D000682:Amyloid / Q000097:blood*; D000686:Amyloidosis / Q000097:blood* / Q000150:complications / Q000473:pathology; D005260:Female; D006801:Humans; D008875:Middle Aged; D012189:Retrospective Studies; D012859:Sjogren's Syndrome / Q000097:blood* / Q000150:complications / Q000473:pathology; D012871:Skin Diseases / Q000097:blood* / Q000150:complications / Q000473:pathology
chemical_list: D000682:Amyloid; C035222:amyloid protein AL