| Bullous pemphigoid (BP) is an autoimmune subepidermal blistering disease characterized by deposition of IgG and or C3 at the basement membrane zone (BMZ) of the epidermis. In most patients with BP, circulating IgG autoantibodies directed against components of hemidesmosomes, 230KD BP antigen 1 (BPAG1), and /or 180 KD BP antigen 2 (BPAG2) are detected. A recent study showed that patients with BP exhibit autoreactive T cell responses to the putative autoantigen BPAG2. The production of humoral or cell-mediated immunity requires the assistance of CD4+ T helper cells. T helper cells are activated by antigens presented in association with calssâ…¡major histocompatibility complex (MHC) molecules, which are encoded by the genes of the HLA-DR,DQ and DP regions on the short arm of chromosome 6. They are normally expressed on the surface of B lymphocytes and antigen-presenting cells of the immune system.Previously, many autoimmune disorders, including pemphigus, have been shown to have specific HLA class â…¡ associations. There have only been a few studies of HLA associations in patients with BP. However, whether Chinese patients with BP are associated with HLA remains to be studied.In this study, 56 southern Chinese BP patient were studied for HLA class â…¡ DRB1,DQA1,DQB1 alleles, using the polymerase chain reaction sequence specific oligonucleotide probes method; Western blot method were also employed to detect autoantigens in the epidermis; Correlattion between clinical features and HLA-DR,DQ genes of BP patients were also analysed.There is significant increase of HLA-DRB1*1001 in BP group, when compared to control subjects; there is also increase frequency of DRB1*1001-DQB1*0501 in BP group; DRB1*1001 has a positive correlation with mucus involvement.HLA-DRB1*04 linked with DQB1*0302, has positive correlationwith BP 180 antigen but negative with itch.HLA-DRB1*12 has decrease frequency in BP group, has negative correlation with age and glucocorticoid hormone therapy, suggesting that it might be a protective gene.Autoantigen BP230, BP230/180 and BP230/180 combined with minor antigen involved in acute phase of BP patient; While BP180 involved mainly in the remission of the disease.In conclusion, there are correlations between HLA class â…¡ genes with clinical features and autoantigens of bullous pemphigoid. |
