Sjögren's syndrome and localized nodular cutaneous amyloidosis: coincidence or a distinct clinical entity?
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.
Meijer
Jiska M
JM
University of Groningen, Groningen, The Netherlands.
Schonland
Stefan O
SO
Palladini
Giovanni
G
Merlini
Giampaolo
G
Hegenbart
Ute
U
Ciocca
Olga
O
Perfetti
Vittorio
V
Leijsma
Martha K
MK
Bootsma
Hendrika
H
Hazenberg
Bouke P C
BP
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
D000682:Amyloid; C035222:amyloid protein AL