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.

Three cases of localized cutaneous nodular amyloidosis in patients with limited systemic sclerosis and a brief literature review

“…Localized cutaneous nodular amyloidosis (LCNA) is the rarest form of localized cutaneous amyloidosis (Summers and Kendrick 2008) and may be associated with autoimmune connective tissue diseases such as Sjögren syndrome (SS; Meijer et al 2008) and limited systemic sclerosis (LSSc; formerly known as CREST syndrome; Damian and Bertouch, 2010, Shiman et al, 2010, Summers and Kendrick, 2008). We report three cases of LCNA in patients with LSSc and briefly review existing information in current literature.…”

Multiple lesions of primary cutaneous nodular amyloidosis in Sjögren syndrome

“…2 It may be hypothesized that the presence of plasma cells in lesions of nodular amyloidosis in patients with SS is a result of homing of these cells to the skin, akin to the presence of plasma cells in other epithelia of patients with SS such as oral and ocular mucosa. 3,4 Plasma cells in nodular amyloidosis have been reported to be clonal and may be viewed as part of the spectrum of the lymphoproliferative disorders associated with SS. 5 The plasma cells in our patient's skin biopsy specimen did not reveal kappa or lambda light chain restriction, excluding a lymphoproliferative process.…”

“…1 Demodex mites belong to the phylum Arthropoda and are known inhabitants of the pilosebaceous units in mammals. 2,3 Of the Demodex species known to inhabit mammals, D folliculorum and D brevis are the only species documented to populate pilosebaceous units in human beings. 1 …”

Amyloidosis

“…5 Localized nodular skin amyloidosis is sometimes associated with Sjö gren disease. 6 In contrast to localized amyloidosis, systemic amyloidosis leads to serious signs and symptoms caused by progressive disease in organs and tissues. There are many types of systemic amyloidosis (see Table 1), but 4 types are seen most frequently: AL, AA, ATTR, and Ab 2 M amyloidosis.…”