The recent availability of cDNA clones for pemphigus antigens has allowed the production of recombinant desmoglein 1 and desmoglein 3 molecules and the development of an ELISA approach in order to determine levels of antibodies to them. The aim of the study was to determine the relationship between autoantibodies levels and the extent of both mucosal and skin lesions in 20 patients with pemphigus vulgaris at the time of diagnosis and during follow-up. For the detection of autoantibodies by ELISA we used the recombinant proteins expressing overlapping sequences with the entire extracellular desmoglein 1 and desmoglein 3 domains. We showed that in presence of mucosal lesions there was a correlation between extension of mucosal involvement and autoantiboidies titres against both desmoglein 1 and desmoglein 3, whereas in presence of skin lesions there was a statistically significant correlation between extension of skin lesions and autoantibodies titres against desmoglein 3, but not against desmoglein 1. A not negligible number of patients showed variations of the desmoglein 3 autoantibodies titre which did not correlate with the severity of both cutaneous and mucosal involvement. Similar results were obtained analyzing autoantibodies titres against desmoglein 1. In conclusion, we believe that the utilization of recombinant desmoglein 1 and desmoglein 3 proteins by ELISA should be used with caution to monitor disease severity and response to therapy, although it remains a high specific test for the initial diagnosis of pemphigus and the identification of a change in the clinical phenotype of this condition.

CSS has shown to be a reliable method to assess the skin lesions in patients with LS. It is reproducible, easy to use and, with the support of the CSS software, applicable worldwide.

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The skin immune system is characterized by the presence of two types of CD1a expressing cells: Langerhans cells and dermal dendritic cells, which are professional antigen processing and presenting cells. It is well established that several dermatoses are associated with T-cell mediated immune responses. In these pathological skin conditions, T-cells are activated by professional antigen presenting cells and dendritic cells are the most potent antigen presenting cells for both Thelper cells and T-cytotoxic cells. Therefore, it is plausible that dendritic cells are crucially involved in the pathogenesis of lymphoproliferative skin conditions characterized by the presence of a T-cell infiltrate. In this study, we examined the frequency and distribution of CD1a expressing cells and CD3 + cells in both the dermal and epidermal compartment in a wide range of lymphoproliferative dermatoses with a T-lymphoid infiltrate. In the skin conditions investigated, the CD1a molecule was highly expressed in mycosis fungoides, T-cutaneous lymphoid hyperplasia, lymphomatoid papulosis and parapsoriasis, whereas few CD1a-positive cells were observed in cutaneous B-cell lymphomas. Our study demonstrates a distinct pattern of CD1a staining in the infiltrates of cutaneous lymphoproliferative disorders which may reflect different immunoregulatory events involving T-lymphocytes and CD1a-positive dermal and epidermal dendritic cells.

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