Inflammatory cytokine expression around the blister could play a mediator role in pemphigus lesions by increasing epithelial damage.

This paper presents the morphological evidence that apoptosis and acantholysis are linked. Therefore, the Fas pathway is associated with CD8 cells in pemphigus lesions.

In normal conditions, the intracellular autoantigens reach the cell surface by apoptosis and are normally cleared by phagocytes without inflammation, nevertheless the lack of depuration of apoptotic material foster the autoantibody production in individuals genetically predisposed, equally defects in signaling, execution and malfunction of the apoptotic pathways may induce autoimmunity, in consequence apoptosis is another way of understanding the autoimmunity (1)..

Scleroderma is an autoimmune disease characterized by early inflammatory infiltrates followed by fibrosis in the skin and internal organs. CREST is a relatively benign cutaneous variant of scleroderma that features calcinosis, Raynaud's phenomenon, oesophageal dysfunction, sclerodactyly and telangiectases. Glomerulonephritis is a rare association of CREST. We are reporting a patient with CREST who developed glomerulonephritis and had anticentromere and antineutrophil cytoplasmic autoantibodies (ANCA) in her serum.

The cellular localization of the Ro/SS-A antigen was defined using indirect immunofluorescence and eight monospecific anti- Ro/SS-A antisera which were identified by immunoblotting. Several mammalian tissues were used as substrates. The Ro/SS-A antigen was located mainly in the nucleus of dog liver and Hep-2-cells, and anti-Ro sera had produced a speckled staining pattern. Cytoplasmic fluorescence appeared only in one serum. IgG was the predominant immunoglobulin with anti-Ro/SS-A activity; five sera had complement fixing activity. The sera absorption studies with partially purified Ro/SS-A antigen and the negativation of the ANA tests had confirmed the specificity of our findings.

Theoretically, the immunization of experimental animals with an anti-idiotype antibody may elicit antibodies that recognize epitopes like the original idiotype; this is archived via internal images. Using this strategy, we attempted to produce anti-epithelial antibodies in Balb/c mice immunized with a pemphigus anti-idiotypic determinant. First, when an anti-idiotype antibody was produced in rabbits by immunization with pemphigus immunoglobulin G (IgG), the anti-idiotypic activity was tested successfully. The anti-idiotype IgG was digested with pepsin and purified by gel filtration chromatography to obtain F(ab')(2) fragments, which were used to immunize Balb/c mice. A control group was immunized with normal IgG. The experimental animals immunized with anti-idiotype F(ab')(2) fragments developed anti-epithelial antibodies in the following two months. The elicited antibodies had anti-desmoglein 1 specificity. Additionally, the skin biopsies of these animals exhibited antibody deposition along intercellular spaces of epidermis, and 25% of them developed blisters. Sera and skin biopsies of control Balb/c mice group were negative. In conclusion, the immunization with pemphigus anti-idiotype antibody may elicit anti-epithelial antibodies via internal images. This experimental approach can be used to understand the pathogenic mechanisms of pemphigus.

Aim:  The present study addresses the question whether nitric oxide (NO) plays a role in inflammation of salivary glands in Sjögren's syndrome. Methods:  The expression of inducible nitric oxide synthase (iNOS) and the presence of citrulline, as a monitor of NO activity were studied by immunohistochemistry, in minor salivary glands from 24 patients with primary Sjögren syndrome. An equal number of control tissues were included. The presence of mRNA of eNOS (endothelial NOS) and iNOS in tissues was studied by fluorescent in situ hybridization. Results:  All salivary glands displayed eNOS along the ductal epithelia, blood vessels and acini. In Sjögren disease iNOS enzyme was widely expressed along ductal epithelia, acini and in foci of lymphocyte infiltration; therefore by the extensive citrulline presence and iNOS, we infer that NO is related to inflammation. Control biopsies were negative for iNOS and citrulline. Conclusion:  The present data suggest that local production of NO should contribute to salivary gland inflammation in Sjögren disease.

Fas ligand (L) is a membrane protein from the tumor necrosis factor (TNF) family. It induces apoptosis upon contact with its Fas/CD95/APO1 receptor. Trimerization of FasL on the surface of effector cells is essential in the binding of the Fas trimer of the target cells. The receptor then recruits an adaptor and caspase-like proteins which lead apoptosis. This paper reports on the fate of FasL in HEp-2 cells committed to apoptosis by induction with campthotecin. Our main results demonstrated that in non-apoptotic cells, FasL aggregates in the cytoplasm forming trimers of 120 kDa. Apoptosis increases the trimeric FasL species, but also induces its dissociation into monomers of 35 kDa. In conclusion, camptothecin appears to perturb the Fas and FasL segregation in the cytoplasm by promoting the transit of FasL to the cell surface, thus fostering a process of autocrine or paracrine apoptosis. FasL is trimerized prior to Fas/FasL complex formation, and after apoptosis, FasL undergoes an intense turnover.