Sjogren’s syndrome (SS) is a systemic autoimmune exocrinopathy that affects the salivary and lacrimal glands, it typically presents as the “sicca complex” of dry eyes (xerophthalmia) and dry mouth (xerostomia) along with other symptoms such as arthritis. SS is classified as either primary or secondary. In the primary form, dry eyes and dry mouth occur alone. In the secondary form, the dry eyes and dry mouth occur in the context of another rheumatic disease, most commonly rheumatoid adhritis. There is an increasing list of systemic manifestations affecting the lung, kidney, and nervous system in patients with SS. The skin is affected in half of SS patients. Despite this high frequency of cutaneous involvement, patients with SS are not commonly seen in dermatology practices. SS is underrecognized and underdiagnosed because the cutaneous manifestations are nonspecific (eg, xerosis, pruritus) and less severe than the oral, ocular, or musculoskeletal symptoms. Nonetheless, because of its high prevalence, risk of cutaneous vasculitis, and the increased risk of a lymphoproliferative disorder, it is important for dermatologists to be familiar with SS.

Sjogren’s syndrome (SS) is a systemic autoimmune exocrinopathy that affects the salivary and lacrimal glands, it typically presents as the “sicca complex” of dry eyes (xerophthalmia) and dry mouth (xerostomia) along with other symptoms such as arthritis.