In primary Sjögren's syndrome, various cutaneous manifestations may become apparent: annular erythema or eruptions reminiscent of erythema multiforme, lichen planus or erythema nodosum (dermo-panniculitis), chilblain-like erythema, purpura, cheilitis, and xerosis, are some of them. In the clinical diagnosis, the following laboratory tests are of value: Schirmer test, rose bengal test, fluorescence staining techniques, gum test, sialography, sialoscintigraphy, lip biopsy. In some cases, dryness can be absent though the above-mentioned skin manifestations are observed. Mothers with Sjögren's syndrome can deliver babies with neonatal lupus erythematosus. Serological testing almost always reveals anti-Ro/SSA-autoantibody and the more specific anti-La/SSB in the sera of patients. The sensitivity of detection can be enhanced by using the immunoblotting technique and the two-dimensional immunoblotting method. In some cases, a new antibody against carbonic anhydrase has been detected.