[Analysis of immunological mechanisms in the pathogenesis of generalized pustular psoriasis]

Generalized pustular psoriasis is one of life-threatening skin diseases which represents sudden onset of severe systemic symptoms such as high fever and chill as well as burning erythema and aseptic pustules over the entire skin. Although the precise mechanism of this rare disease is unknown, several lines of clinical and experimental observations have suggested that certain immunological mechanisms play important roles in the pathogenesis of this disease. In this study, in order to attain better understanding of the immunological events involved in generalized pustular psoriasis, several in vivo and in vitro immunological experiments have been performed. The results obtained are as follows: 1) sera from patients contained high amount of inflammatory cytokines, 2) peripheral blood mononuclear cells(PBMC) showed high proliferative responses to bacteria-derived super antigens, 3) PBMC from patients produced a large amount of cytokines when stimulated by mitogens in vitro, 4) endothelial cells in the lesional skins of patients exhibited enhanced expression of adhesion molecules, and 5) the expressions of these adhesion molecules on human endothelial cells were differently regulated by several cytokines. These results suggest that the activation of peripheral blood mononuclear cells by bacteria-derived super antigens followed by cytokine production, and the induction of the adhesion molecules expressions on the endothelial cells are important immunological events in the forming the characteristic clinical symptoms of generalized pustular psoriasis.