Cutaneous amyloidosis with Sjögren syndrome a distinct disease entity?

Meijer JM, Schonland SO, Palladini G, et al, University of Groningen, Groningen, the Netherlands, and other centers. Sjgren's syndrome and localized nodular cutaneous amyloidosis: coincidence or a distinct clinical entity? Arthritis Rheum. 2008;58:1992-1999.

Although localized nodular cutaneous amyloidosis is rare,many reported cases are related to Sjgren syndrome (SS). Therefore, looking for signs of SS in patients with cutaneous amyloidosis is useful.

Meijer and associates retrospectively searched amyloidosis center patient registries for patients who had the combination of cutaneous localized amyloid and SS; 8 patients (all women) were identified. The current American-European Consensus Group (AECG) criteria were used to determine how many cases adhered to the current definition of SS. Histopathological data and information about recently determined serological parameters were evaluated.

Six patients fulfilled the AECG criteria for SS. All patients had sicca symptoms, 6 had xerostomia, and 7 had xerophthalmia.The presence of the immunoglobulin light chain–associated, or AL,amyloid was confirmed in 4 patients. In 3 of them and in 2 other patients, a light chain-restricted plasma cell population was observed close to the amyloid deposits in the skin. Progression to systemic AL amyloidosis was not observed in any patients during follow-up (median, 3.5 years).

The authors noted that the combination of cutaneous amyloidosis and SS appears to be a distinct disease entity that reflects a particular and benign part of the polymorphic spectrum of lymphoproliferative diseases related to SS.