Sjogren's syndrome (SS) is a systemic autoimmune exocrinopathy that affects
the salivary and lacrimal glands. It typically presents as the "sicca comp
lex" of dry eyes (xerophthalmia) and dry mouth (xerostomia) along with of h
er symptoms such as arthritis. SS is classified as either primary or second
ary. In the primary form, dry eyes and dry mouth occur alone. In the second
ary form, the dry eyes and dry mouth occur in the context of another rheuma
tic disease, most commonly rheumatoid arthritis. There is an increasing lis
t 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 comm
only seen in dermatology practices. SS is underrecognized and underdiagnose
d because the cutaneous manifestations are nonspecific (eg, xerosis, prurit
us) and less severe than the oral, ocular, or musculoskeletal symptoms. Non
etheless, 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. Copyright (C) 2001 by W.B. Saunders
Company.