Dermatologic Manifestations of Sjögren Syndrome

Abstract

Sjögren syndrome (SS) is a chronic autoimmune inflammatory disease that involves primarily the exocrine glands, resulting in their functional impairment. SS typically presents as dry eyes (xerophthalmia) and dry mouth (xerostomia). This process can manifest either as the independent phenomenon of primary SS or as secondary SS when found in the context of another autoimmune process, most commonly rheumatoid arthritis or systemic lupus erythematosus. Nearly half of the patients with SS develop cutaneous manifestations, which may include dry skin (xeroderma), palpable and nonpalpable purpura, and/or urticaria-like lesions. These cutaneous manifestations have been underemphasized because they are often overshadowed by the more prominent sicca symptoms. However, certain skin findings are of paramount clinical and prognostic importance as they confer an increased risk for the development of life-threatening conditions, including multisystem vasculitis and non-Hodgkin lymphoma.OBJECTIVE AND CONCLUSIONS:In this review, the cutaneous manifestations of primary SS are discussed, with an emphasis on those findings that portend an increased risk of mortality.

Pathological Analysis of Ocular Lesions in a Murine Model of Sjögren’s Syndrome

“…The primary clinical symptoms are those of sicca syndrome, including dry eyes (xerophthalmia) and mouth (xerostomia) [4]. Extraglandular cutaneous, pulmonary, gastrointestinal, cardiac, neurological, and renal symptoms may occur in SS patients [13,14,15,16,17]. Keratoconjunctivitis associated with dry eyes is a severe, extremely uncomfortable symptom of this disease.…”

Concomitant Darier′s disease and Sjφgren′s syndrome

“…[3] Sjögren's syndrome is an autoimmune connective disorder characterized by xerophthalmia and xerostomia due to lymphocytic infiltration of the lacrimal and salivary glands. [2] Earlier studies detected mitochondrial alterations in cells from these patients, which were related to the action of antimitochondrial autoantibodies. Recently, Perez et al, found that gene loci such as 1p34.2 (D1S3721), 3q13.31-q13.32 (D3S2460) were differentially expressed in labial salivary glands and epithelial cells in Sjögren's syndrome.…”

“…[1] Sjögren's syndrome is a systemic autoimmune disease in which the exocrine glands are damaged by immune cells. [2] Herein, we describe a patient with the rare coexistence of Darier's disease and Sjögren's syndrome.…”

Primary Sjögren’s Syndrome: Current Pathophysiological, Diagnostic and Therapeutic Advances

“…The symptoms usually resolve with corticosteroids. Other skin manifestations include erythema nodosa, vitiligo, and digital ulcers (Kittridge et al, 2011).…”