[Nodular cutaneous amyloidosis associated with Sjögren's syndrome]. / Amylose cutanée nodulaire et syndrome de Gougerot-Sjögren.

ABSTRACT

BACKGROUND: Cutaneous nodular amyloidosis is rare, and association with Sjögren's syndrome has been reported. We discuss the possible link between these two diseases based on a case we saw. PATIENTS AND METHODS: A 78-year-old woman with Sjögren's syndrome ongoing for 17 years presented for evaluation of a waxy infiltrated lesion on her left leg that had appeared 6 months earlier. Histopathological examination revealed a deposit of homogenous eosinophilic material throughout the dermis consistent with amyloidosis. Immunohistochemical study showed these deposits to be AL immunoglobin light chains. DISCUSSION: The association of cutaneous nodular amyloidosis and Sjögren's syndrome appears to be a distinct disease entity reflecting the polymorphic clinical spectrum of lymphoproliferative diseases related to Sjögren's syndrome.