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Multiple hereditary infundibulocystic basal cell carcinomas: a distinctive genodermatosis

and EM did not show a statistical difference, although the mean value of sCD40L was higher in the former. Our results show that in SJS/TEN there is not only an intralesional involvement, but also a systemic activation of the CD40/CD40L system. In particular, the penetration and the diffusion of the triggering agent in genetically predisposed individuals may induce CD4+ T lymphocytes to release sCD40L. Thus, sCD40L may exert its immunological functions by interacting both with circulating CD40+ cells, amplifying the immune response to the whole mucocutaneous surface, and with immune and nonimmune CD40+ cells localized in tissue, contributing to the development of the lesions. Although our study suggests a role for sCD40L as a marker of activation in SJS/TEN, it was not possible to evaluate whether its serum concentrations reflect disease severity. Nevertheless, our data showed that the patients with wider dermal– epidermal detachment had the highest levels of sCD40L. Furthermore, our results show that sCD40L was expressed to a greater degree in patients with EM than in controls. This finding suggests that this molecule could be considered a marker of activation also in EM, in which environmental agents, in particular viruses and other microorganisms, may induce a dysregulation of systemic immunity. Finally, possible implications regarding the therapy of SJS/ TEN arise from the present study. Because of the severity of the disease and the high mortality rates of SJS/TEN, new therapeutic agents may be researched. CD40/CD40L interactions are thought to play an important role in the pathogenesis of certain diseases, such as systemic lupus erythematosus (SLE), rheumatoid arthritis and systemic sclerosis. Recently, pharmacological manipulation of this axis has been tried, with promising results both in animal models and in human SLE. Although further studies are required, our finding that serum sCD40L concentrations and CD40L+ lesional cells in patients with SJS/TEN are elevated suggests that inhibition of CD40/CD40L interactions could be a therapeutic target in SJS/TEN.