Sjögren's syndrome in dermatology.

Abstract:

:Sjogren's Syndrome (SS) is a systemic autoimmune disease characterized by dry eyes (keratoconjunctivis sicca) and dry mouth (xerostomia). To fulfill diagnostic criteria, patients must have objective signs of dryness on examination and laboratory confirmation of an autoimmune process as evidenced by a positive autoantibody to SS-A antigen or a characteristic lip biopsy. SS may exist as a primary condition or in association with other systemic autoimmune disorders (termed secondary SS) such as rheumatoid arthritis, systemic lupus erythematous (SLE), progressive systemic sclerosis (scleroderma), or dermatomyositis. Exclusions to the diagnosis include pre-existing lymphoma, hepatitis C or HIV infection. Pathogenesis involves both genetic (especially HLA-DR) and environmental factors. Both T-cells and B-cells are involved in the generation of cytokines and chemokines within the glands. The epithelial cells of the glands also play a role in pathogenesis. The dermatologic manifestations range from drynessness (sicca) and its complications to vasculitis. There is a significant overlap in the clinical manifestations, as well as treatment, of SS and SLE. However, SS patients require special attention to the complications of ocular dryness (keratocojunctivitis sicca and blepharitis) and oral dryness (rapid tooth loss and oral candidiasis) SS patients have a markedly increased risk of lymphoma and enlarged lymph nodes or persistently enlarged parotid/submandibular glands that require further evaluation.

journal_name

Clin Dermatol

journal_title

Clinics in dermatology

authors

Fox RI,Liu AY

doi

10.1016/j.clindermatol.2006.07.005

subject

Has Abstract

pub_date

2006-09-01 00:00:00

pages

393-413

issue

5

eissn

0738-081X

issn

1879-1131

pii

S0738-081X(06)00097-6

journal_volume

24

pub_type

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