Pemphigus Pathogenesis

Pemphigus is a chronic skin and mucosal disease in which the cause remains unknown. It is characterized by intra-epidermal acantholysis. Many studies have suggested that it is a disease of multifactorial disorder . Genetic, immunologic and environmental components may be involved in it. The important pathologic role of autoantibodies against desmoglein has been confirmed . However, at what conditions the pemphigus antibodies can be produced ? What's the mechanisms of acantholysis?. We try to make clear the above mentioned questions in our study.The activation of B lymphocytes usually depends on the help of T cells and the activation of T cells is restricted to class II human leukocyte antigen (HLA). Some studies from foreign countries have indicated that pemphigus are associated with HLA . The relationship between HLA and disease has ethnical differences . Few studies have been carried out on the immunogenetic characteristics of the disease in our country . In order to study the etiologic factors of the disease and its susceptible genes , we used polymerase chain reaction - sequence specific primers (PCR-SSP) to determine HLA-DR and DQB1 genes of patients with pemphigus vulgaris (PV) and pemphigus erythematosus (PE) in Han nationalities of Jiangsu and Anhui provinces at population level , and investigated the association of clinical characteristics of PV with HLA class II genes .The pathogenic role of autoantibodies in pemphigus has been confirmed. Deposition of intercellular IgG, however, is not limited to clinically diseased skin. Hence, mechanisms other than antibody deposition appear to be important for blister formation in pemphigus. Some studies have indicated that some cytokines such as tumor necrosis factor-α (TNF-α )are important in the process of acantholysis . For the purpose of exploration the role of some cytokines in pemphigus pathogenesis and their association with the disease condition , we detected interleukin-6 (IL-6) and TNF- α in pemphigus patients using double antibody sandwich enzyme-linked immunosorbent assay (ELISA).I We typed HLA-DR, DQB1 genes in 61 PV, 37 PE patients of Han nationalities and 57 matched control subjects using PCR-SSP. The results demonstrated that DR4, DRB1*14, DQB 1*0503 and DQB 1*0302 gene frequencies were significantly higher in both PV and PE patients than those in controls. Further typing of DR4 positive subjects...