Identical HLA class II alleles predispose to drug-triggered and idiopathic pemphigus vulgaris

In pemphigus vulgaris, a dermatological autoimmune disease, specific human leukocyte antigen (HLA) class II alleles, DR4 (DRB1*0402) and DRw14 (DRB1*1401, in linkage disequilibrium with DQB1*0503), are thought to be susceptibility genes involved in the onset of the disease. We studied the HLA class II alleles (DQA1, DQB1, DRB1 and DPB1) of 6 patients with pemphigus, in whom the disease was 'triggered' by drugs containing sulphydryl or another sulphur-containing group. All patients carried the DRB1*0402 susceptibility allele, and one patient also carried the second susceptibility allele, namely DQB1*0503 (in linkage with DRB1*1401). Bacterial, viral or environmental agents are suspected to trigger the onset of autoimmune diseases. Our study demonstrated the presence in patients with drug-triggered pemphigus vulgaris of the same HLA alleles thought to predispose to idiopathic pemphigus vulgaris. This finding strengthens the notion that these HLA alleles may be true disease susceptibility genes.